UNIT 7 Nursing Management of patients with disorders of blood

🩸 Anatomy and Physiology of Blood

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🔬 Definition of Blood

Blood is a specialized connective tissue composed of cells and plasma. It performs vital transport, regulatory, and protective functions in the body.

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🧪 Composition of Blood

Total blood volume ≈ 5–6 liters (in adults)
🔹 55% Plasma
🔹 45% Formed Elements (Cells)

🔷 1. Plasma (55%)

• 🟡 Straw-colored, non-cellular fluid
• 90–92% water 💧
• 8–10% solutes, including:
  • 🧬 Proteins (Albumin, Globulin, Fibrinogen)
  • 🧂 Electrolytes (Na⁺, K⁺, Cl⁻, HCO₃⁻)
  • 🍬 Nutrients (Glucose, Amino acids)
  • 🧪 Hormones, enzymes
  • ❌ Waste products (Urea, Creatinine)

🔷 2. Formed Elements (45%)

1. 🔴 Red Blood Cells (RBCs / Erythrocytes)
   • Biconcave, non-nucleated
   • Count: ~4.5–6 million/µL
   • Lifespan: ~120 days
   • Contain hemoglobin (Hb) for O₂ transport
     ➤ Hb + O₂ → Oxyhemoglobin
   • Formed in red bone marrow
2. ⚪ White Blood Cells (WBCs / Leukocytes)
   • Nucleated, immune cells
   • Count: 4,000–11,000/µL
   • Types:
     • 🧪 Granulocytes
       ➤ Neutrophils 🦠 (60–70%) – phagocytosis
       ➤ Eosinophils 🪱 (2–4%) – allergy, parasitic defense
       ➤ Basophils 🧨 (<1%) – release histamine
     • 🔬 Agranulocytes
       ➤ Lymphocytes (20–25%) – B & T cells (immunity)
       ➤ Monocytes (3–8%) – become macrophages
3. 🟡 Platelets (Thrombocytes)
   • Small, disc-shaped fragments
   • Count: 150,000–400,000/µL
   • Lifespan: 8–10 days
   • Function: Blood clotting 🩹
     ➤ Help form platelet plug + activate coagulation cascade

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❤️ Functions of Blood

🔢	Function	Description
1️⃣	Transport	🚚 Carries oxygen (O₂), carbon dioxide (CO₂), nutrients, hormones, waste products
2️⃣	Regulation	⚖️ Maintains pH (7.35–7.45), temperature (98.6°F), water-electrolyte balance
3️⃣	Protection	🛡️ WBCs fight infection; platelets + plasma proteins stop bleeding

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🧠 Physiology of Blood

🔁 1. Oxygen & CO₂ Transport

• O₂ from lungs → tissues via Hb
• CO₂ from tissues → lungs via plasma & RBCs

💥 2. Hemostasis (Prevention of Blood Loss)

3 Steps:

1. Vascular spasm – vessel constriction
2. Platelet plug formation
3. Coagulation – clotting cascade → fibrin mesh 🕸️

🔄 3. Immune Response

• WBCs recognize and destroy pathogens
• Lymphocytes produce antibodies
• Monocytes become macrophages

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🧬 Blood Groups & Rh Factor

🔹 ABO System:

• A, B, AB, O types based on antigens on RBCs
• Antibodies present in plasma
  • A → Anti-B
  • B → Anti-A
  • AB → None
  • O → Anti-A & Anti-B

🔹 Rh Factor:

• Rh⁺ = has antigen
• Rh⁻ = no antigen
  Important in pregnancy & transfusions 🤰💉

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🔄 Blood Circulation

🫀 Heart → Arteries → Capillaries → Veins → Heart

• Arteries carry oxygenated blood (except pulmonary artery)
• Veins carry deoxygenated blood (except pulmonary vein)
• Capillaries are exchange sites

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🧫 Hematopoiesis (Blood Cell Formation)

• Occurs in red bone marrow
• Stem cells (pluripotent) differentiate into:
  • ➤ RBCs (erythropoiesis)
  • ➤ WBCs (leukopoiesis)
  • ➤ Platelets (thrombopoiesis)

Hormonal control:

• Erythropoietin (from kidneys) stimulates RBC production

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🧠 Key Points to Remember

✔️ Blood = 8% of body weight
✔️ RBCs carry O₂ via Hb
✔️ WBCs = body’s defense army
✔️ Platelets = clotting agents
✔️ Plasma = carrier of nutrients, hormones, waste
✔️ ABO & Rh system are essential in transfusion safety

🧪 Common Blood Tests: Normal Ranges & Interpretation

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🩸 1. Complete Blood Count (CBC)

🔬 Test	📊 Normal Range	🧠 Interpretation
Hemoglobin (Hb)	♂ 13–17 g/dL ♀ 12–15 g/dL	🔽 Low: Anemia, bleeding, malnutrition 🔼 High: Dehydration, polycythemia
Hematocrit (Hct)	♂ 40–50% ♀ 36–44%	🔽 Low: Anemia, overhydration 🔼 High: Dehydration, polycythemia
RBC Count	♂ 4.5–6.0 million/µL ♀ 4.0–5.5 million/µL	🔽 Low: Anemia 🔼 High: Polycythemia
WBC Count	4,000–11,000 /µL	🔽 Low: Bone marrow suppression, viral infection 🔼 High: Infection, inflammation, leukemia
Platelets	150,000–400,000 /µL	🔽 Low: Thrombocytopenia (bleeding risk) 🔼 High: Thrombocytosis (clotting risk)
MCV (Mean Corpuscular Volume)	80–100 fL	🔽 Microcytic anemia 🔼 Macrocytic anemia
MCH/MCHC	MCH: 27–31 pg MCHC: 32–36%	Altered in various types of anemia

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🧪 2. Blood Glucose Tests

🔬 Test	📊 Normal Range	🧠 Interpretation
Fasting Blood Sugar (FBS)	70–100 mg/dL	🔽 Hypoglycemia 🔼 Diabetes mellitus
Postprandial (PPBS)	<140 mg/dL (2 hrs after meal)	🔼 Diabetes, impaired glucose tolerance
Random Blood Sugar (RBS)	<140 mg/dL	🔼 Diabetes suspicion
HbA1c (Glycated Hemoglobin)	4–5.6% (normal) 5.7–6.4% (pre-diabetes) ≥6.5% (diabetes)	Reflects average blood sugar for last 2–3 months

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⚖️ 3. Liver Function Test (LFT)

🔬 Test	📊 Normal Range	🧠 Interpretation
ALT (SGPT)	7–56 U/L	🔼 Liver damage, hepatitis
AST (SGOT)	10–40 U/L	🔼 Liver or heart disease
ALP (Alkaline Phosphatase)	44–147 U/L	🔼 Bone/liver disorder
Total Bilirubin	0.2–1.2 mg/dL	🔼 Jaundice, liver disease
Direct Bilirubin	0.1–0.3 mg/dL	🔼 Obstructive jaundice
Albumin	3.5–5.5 g/dL	🔽 Liver/kidney disease
Total Protein	6–8 g/dL	🔽 Liver/kidney problems 🔼 Chronic inflammation

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🧂 4. Kidney Function Test (KFT / RFT)

🔬 Test	📊 Normal Range	🧠 Interpretation
Serum Creatinine	♂ 0.7–1.3 mg/dL ♀ 0.6–1.1 mg/dL	🔼 Renal impairment
Blood Urea Nitrogen (BUN)	7–20 mg/dL	🔼 Dehydration, renal dysfunction
Uric Acid	♂ 3.5–7.2 mg/dL ♀ 2.6–6.0 mg/dL	🔼 Gout, kidney disease
eGFR	>90 mL/min/1.73m²	🔽 Reduced in chronic kidney disease

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⚙️ 5. Lipid Profile

🔬 Test	📊 Normal Range	🧠 Interpretation
Total Cholesterol	<200 mg/dL	🔼 Risk of atherosclerosis
LDL (“bad”)	<100 mg/dL	🔼 Increased heart disease risk
HDL (“good”)	♂ >40 mg/dL ♀ >50 mg/dL	🔽 Risk of heart disease
Triglycerides	<150 mg/dL	🔼 Pancreatitis, heart disease

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🧪 6. Thyroid Function Test (TFT)

🔬 Test	📊 Normal Range	🧠 Interpretation
TSH (Thyroid Stimulating Hormone)	0.4–4.0 mIU/L	🔽 Hyperthyroidism 🔼 Hypothyroidism
T3 (Triiodothyronine)	80–200 ng/dL	🔼 Hyperthyroidism 🔽 Hypothyroidism
T4 (Thyroxine)	5.0–12.0 µg/dL	Same as above

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🧬 7. Coagulation Profile

🔬 Test	📊 Normal Range	🧠 Interpretation
Prothrombin Time (PT)	11–13.5 sec	🔼 Bleeding risk (e.g., liver disease, warfarin)
INR	0.8–1.1 (normal) 2–3 (therapeutic)	🔽 Risk of clot 🔼 Risk of bleeding
aPTT	25–35 sec	🔼 Hemophilia, heparin therapy

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🧪 8. Electrolytes & Minerals

🔬 Test	📊 Normal Range	🧠 Interpretation
Sodium (Na⁺)	135–145 mEq/L	🔼 Dehydration 🔽 Diuretics, Addison’s
Potassium (K⁺)	3.5–5.0 mEq/L	🔽 Muscle cramps, arrhythmias 🔼 Renal failure
Calcium (Ca²⁺)	8.5–10.5 mg/dL	🔼 Hyperparathyroidism 🔽 Tetany, vitamin D deficiency
Chloride (Cl⁻)	98–106 mEq/L	Electrolyte imbalance
Magnesium (Mg²⁺)	1.7–2.2 mg/dL	🔼 Renal failure 🔽 Neuromuscular irritability

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🧪 9. Infection Markers

🔬 Test	📊 Normal Range	🧠 Interpretation
CRP (C-Reactive Protein)	<10 mg/L	🔼 Inflammation, infection
ESR (Erythrocyte Sedimentation Rate)	♂ <15 mm/hr ♀ <20 mm/hr	🔼 Chronic inflammation
Procalcitonin	<0.1 ng/mL	🔼 Bacterial sepsis
Widal Test / Dengue / Malaria / HIV	Based on titers / +ve/-ve	Detect specific infections

🩸 Nursing Assessment of Patients with Blood Disorders

Blood disorders include anemia, leukemia, hemophilia, thalassemia, polycythemia, DIC, and other hematological conditions. A structured nursing assessment is essential for early diagnosis, intervention, and care planning.

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🗂️ I. Health History Collection (Subjective Data)

🧑‍⚕️ A. Personal Data

• Name, age, gender
• Occupation (exposure to chemicals/radiation?)
• Residence (endemic areas for thalassemia, malaria)

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🩺 B. Chief Complaints

Ask about duration, onset, and severity of:

• 🔻 Fatigue, weakness
• 🧘 Dizziness, fainting
• ❄️ Cold intolerance
• 💨 Shortness of breath on exertion
• 💓 Palpitations or rapid heartbeat
• 🟨 Yellowing of eyes/skin (jaundice)
• 🩹 Easy bruising, bleeding gums, nosebleeds
• 📉 Weight loss, fever, night sweats (esp. in leukemia)

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🧬 C. Past Medical History

• Previous diagnosis of anemia, bleeding disorders, cancer
• History of radiation or chemotherapy
• Frequent infections, past transfusions
• Chronic diseases: kidney failure, liver disease

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🧬 D. Family History

• Hereditary conditions:
  • ➤ Thalassemia, Sickle Cell Anemia
  • ➤ Hemophilia, Leukemia
• Any consanguineous marriage history

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🍎 E. Dietary History

• Adequate iron, folic acid, and vitamin B12 intake?
• Vegetarian/vegan diet?
• Alcohol use (can affect bone marrow)
• Pica (eating clay/ice – common in iron-deficiency anemia)

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💊 F. Medication History

• Anticoagulants (e.g., warfarin)
• Chemotherapy drugs
• NSAIDs (can cause bleeding)
• Iron or vitamin supplements

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💉 G. Blood Transfusion History

• Number of transfusions
• History of transfusion reactions

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🧍‍♀️ II. Physical Assessment (Objective Data)

Perform head-to-toe examination with special focus on signs of hematologic abnormalities.

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🔍 A. General Appearance

• Pallor (skin, conjunctiva, nail beds)
• Fatigue, lethargy
• Underweight or cachexia

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🧑‍🦲 B. Skin, Hair, and Nails

• Pallor, jaundice, cyanosis
• Petechiae (tiny red/purple spots), ecchymosis (bruises)
• Dry, brittle hair (nutritional deficiency)
• Spoon-shaped nails (koilonychia in iron-deficiency anemia)

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👄 C. Head, Eyes, Ears, Nose, Throat

• Pale conjunctiva (anemia)
• Icterus (yellow sclera – hemolytic anemia)
• Bleeding gums or oral ulcers (leukemia, thrombocytopenia)
• Epistaxis (nosebleeds)
• Swollen lymph nodes in neck (lymphoma, leukemia)

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❤️ D. Cardiovascular System

• Tachycardia, palpitations
• Murmurs (in severe anemia)
• Hypotension (if bleeding/hemorrhage)
• Distended neck veins (in polycythemia vera)

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💨 E. Respiratory System

• Tachypnea
• Shortness of breath on mild exertion
• Crackles (if fluid overload due to transfusion reaction)

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🧠 F. Neurological System

• Headache, dizziness
• Confusion, altered sensorium (especially in DIC or severe anemia)
• Tingling or numbness (B12 deficiency)

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🧍 G. Musculoskeletal

• Bone tenderness or pain (in leukemia)
• Joint swelling, bleeding into joints (hemophilia)

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🧫 H. Abdominal Examination

• Hepatosplenomegaly (seen in leukemia, thalassemia, hemolytic anemias)
• Abdominal distension
• Palpable spleen or liver

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🚻 I. Genitourinary

• Hematuria (blood in urine)
• Menorrhagia (excessive menstrual bleeding)
• Reduced urine output (if renal involvement or DIC)

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🌡️ J. Temperature

• Low-grade fever (chronic leukemia)
• Recurrent infections

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📝 III. Investigations to Support Assessment

• CBC: Hb, RBC, WBC, Platelets
• Peripheral smear
• Reticulocyte count
• Iron studies, B12, Folate levels
• Coagulation profile: PT, aPTT, INR
• Bone marrow biopsy (in suspected leukemia/aplastic anemia)
• Genetic testing (for thalassemia, sickle cell)
• Liver & Kidney Function Tests

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🧠 IV. Summary of Key Nursing Assessment Priorities

🧭 Focus Area	🔍 What to Assess
Oxygenation	Breathlessness, cyanosis, O₂ saturation
Perfusion	Capillary refill, HR, BP, pallor
Bleeding risk	Petechiae, bruising, bleeding sites
Infection risk	Fever, lymphadenopathy, WBC count
Fatigue & Nutrition	Activity tolerance, diet recall
Pain	Bone, joint, or abdominal pain
Psychosocial	Anxiety, depression (especially in chronic diseases)

🧪 Diagnostic Tests for Patients with Blood Disorders

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🩸 1. Complete Blood Count (CBC)

🔬 Test Component	📊 Normal Range	🧠 Purpose & Interpretation
Hemoglobin (Hb)	♂ 13–17 g/dL ♀ 12–15 g/dL	Detects anemia or polycythemia
Hematocrit (Hct)	♂ 40–50% ♀ 36–44%	Indicates RBC concentration
RBC count	♂ 4.5–6 million/µL ♀ 4–5.5 million/µL	🔽 in anemia, 🔼 in polycythemia
WBC count	4,000–11,000/µL	🔼 Infection/leukemia, 🔽 bone marrow suppression
Platelet count	150,000–400,000/µL	🔽 Thrombocytopenia, 🔼 clotting disorders
MCV	80–100 fL	Assesses type of anemia (micro/macrocytic)
MCH/MCHC	MCH: 27–31 pg MCHC: 32–36%	Helps differentiate hypo/hyperchromic anemias

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🔬 2. Peripheral Blood Smear (PBS)

🧪 Purpose:

• Visual evaluation of shape, size, and type of blood cells under a microscope

🔍 Findings in Blood Disorders:

• Sickle-shaped cells → Sickle cell anemia
• Target cells → Thalassemia, liver disease
• Howell-Jolly bodies → Asplenia
• Atypical lymphocytes → Leukemia or infections
• Schistocytes (fragmented RBCs) → DIC, hemolysis
• Blast cells → Leukemia
• Hypersegmented neutrophils → B12/Folate deficiency

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🧬 3. Reticulocyte Count

📊 Normal Range: 0.5%–2.5%

🧪 Purpose:

• Measures young (immature) RBCs to assess bone marrow activity

🔍 Interpretation:

• 🔼 in hemolytic anemia or blood loss
• 🔽 in aplastic anemia or marrow failure

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🧫 4. Bone Marrow Aspiration & Biopsy

🧪 Purpose:

• Assess marrow cellularity, detect leukemia, myelodysplastic syndromes, aplastic anemia, myeloma

📍 Common Sites:

• Posterior iliac crest (in adults)
• Sternum (rare)

🔍 Findings:

• Hypocellular marrow → Aplastic anemia
• Hypercellular with blasts → Leukemia
• Increased plasma cells → Multiple myeloma

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🧪 5. Coagulation Profile

Test	Normal Range	Interpretation
PT (Prothrombin Time)	11–13.5 sec	🔼 in liver disease, DIC, warfarin use
INR	0.8–1.1 (normal), 2–3 (therapeutic)	Monitors warfarin
aPTT (Activated Partial Thromboplastin Time)	25–35 sec	🔼 in hemophilia, heparin therapy
Bleeding Time (BT)	2–7 min	🔼 in platelet disorders
Clotting Time (CT)	8–15 min	🔼 in hemophilia, severe thrombocytopenia

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🧪 6. Iron Studies (for Iron-Deficiency or Overload)

Test	Normal Range	Interpretation
Serum Iron	60–170 µg/dL	🔽 in iron-deficiency anemia
Ferritin	♂ 24–336 ng/mL ♀ 11–307 ng/mL	🔽 in iron-deficiency, 🔼 in hemochromatosis
TIBC (Total Iron Binding Capacity)	240–450 µg/dL	🔼 in iron deficiency
Transferrin Saturation	20–50%	🔽 in iron-deficiency anemia

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🧬 7. Vitamin B12 & Folate Levels

Test	Normal Range	Interpretation
Serum B12	200–900 pg/mL	🔽 → Macrocytic/megaloblastic anemia
Folic Acid	2.7–17.0 ng/mL	🔽 → Megaloblastic anemia

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🧪 8. Hemoglobin Electrophoresis

🧪 Purpose:

• Identifies abnormal hemoglobin variants (e.g., HbS, HbC, HbF)

🔍 Used for:

• Sickle cell disease
• Thalassemia (HbA2, HbF elevated)
• Hemoglobinopathies

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🧬 9. Genetic Testing / Molecular Studies

🧪 Purpose:

• Confirms inherited blood disorders

✅ Tests:

• HBB gene mutation (for sickle cell, thalassemia)
• Factor VIII or IX gene (hemophilia A/B)
• JAK2 mutation → Polycythemia vera, myeloproliferative disorders

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🧪 10. LDH (Lactate Dehydrogenase)

| Normal Range: 140–280 U/L |

🔍 High in:

• Hemolysis, Leukemia, Lymphoma, Multiple Myeloma

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🧪 11. Haptoglobin

| Normal Range: 30–200 mg/dL |

🔍 Low in:

• Hemolytic anemias (due to binding free hemoglobin)

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🧪 12. Direct & Indirect Coombs Test

🔍 Detects:

• Autoimmune hemolytic anemia
• Hemolytic disease of newborn
• Incompatibility reactions in transfusions

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🧪 13. Serum Protein Electrophoresis (SPEP)

🧪 Purpose:

• Detects abnormal monoclonal protein (M protein) in:
  • Multiple Myeloma
  • Waldenström’s macroglobulinemia

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🧪 14. Flow Cytometry

🧪 Purpose:

• Classifies types of leukemia or lymphoma
• Measures CD markers on cell surface

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🧪 15. ESR (Erythrocyte Sedimentation Rate) & CRP

| ESR Normal: ♂ <15 mm/hr<br>♀ <20 mm/hr | | CRP: <10 mg/L |

🔍 Interpretation:

• 🔼 in chronic inflammation, infection, leukemia, autoimmune disorders

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🧪 16. D-Dimer Test

| Normal: <500 ng/mL |

🔍 High in:

• Disseminated Intravascular Coagulation (DIC)
• Deep Vein Thrombosis (DVT)
• Pulmonary Embolism (PE)

🩸 ANEMIA

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📘 Definition of Anemia

Anemia is a condition in which the number of red blood cells (RBCs) or the hemoglobin (Hb) concentration in the blood is lower than normal, resulting in a reduced oxygen-carrying capacity of the blood.

🧪 WHO Criteria for Anemia (Hemoglobin Level):

• ♂ <13 g/dL
• ♀ <12 g/dL
• Children (6 months–6 years): <11 g/dL

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⚠️ Causes of Anemia (Etiology)

Anemia can result from one or more of the following three basic mechanisms:

1️⃣ Decreased RBC Production

Occurs when the bone marrow fails to produce enough red blood cells.

🔹 Nutritional Deficiencies:

• Iron deficiency (most common)
• Vitamin B12 deficiency
• Folic acid deficiency

🔹 Bone Marrow Disorders:

• Aplastic anemia
• Leukemia
• Myelodysplastic syndromes

🔹 Chronic Diseases:

• Chronic kidney disease (↓ erythropoietin)
• Liver disease
• Cancer
• Rheumatoid arthritis, tuberculosis

🔹 Endocrine Disorders:

• Hypothyroidism
• Addison’s disease

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2️⃣ Increased RBC Destruction (Hemolysis)

Occurs when red blood cells are destroyed faster than they are produced.

🔹 Hemolytic Anemias:

• Autoimmune hemolytic anemia
• G6PD deficiency
• Hereditary spherocytosis

🔹 Hemoglobinopathies:

• Sickle cell anemia
• Thalassemia

🔹 Infections:

• Malaria
• Septicemia

🔹 Toxins / Drugs:

• Snake venom
• Certain antibiotics, antimalarials

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3️⃣ Blood Loss (Hemorrhagic Anemia)

🔹 Acute Blood Loss:

• Trauma
• Surgery
• Gastrointestinal bleeding (peptic ulcer, hemorrhoids)

🔹 Chronic Blood Loss:

• Menorrhagia (heavy menstrual bleeding)
• Gastric ulcers
• Hookworm infestation
• Malignancies (colon cancer)

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🔄 Multifactorial Causes

In many patients, especially the elderly or chronically ill, anemia may be due to a combination of:

• Nutritional deficiency
• Chronic disease
• Inflammation or malignancy

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📌 Summary Table: Causes of Anemia

🧭 Category	🔍 Example Causes
↓ Production	Iron, B12, Folate deficiency, CKD, marrow failure
↑ Destruction	Hemolysis, Sickle cell, Thalassemia, infections
Blood Loss	Trauma, GI bleed, menstruation, parasitic infection

🩸 TYPES OF ANEMIA

Anemias are broadly classified based on:

1. 🧪 Etiology (cause) – what leads to anemia
2. 🧬 Pathophysiology – how the disease affects RBCs
3. 🔬 Morphology – size (MCV) and color (MCHC) of RBCs

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🧪 I. Based on Etiology (Cause)

1️⃣ Anemia due to Decreased RBC Production

A. Iron Deficiency Anemia

• 🔻 Iron → 🔻 Hemoglobin synthesis
• 🧾 Causes: Poor diet, chronic blood loss (GI bleed, menstruation), malabsorption
• 🩸 Microcytic, hypochromic

B. Megaloblastic Anemia

• 🔻 DNA synthesis due to B12 or Folate deficiency
• 🧾 Causes: Poor intake, malabsorption (pernicious anemia), alcoholism
• 🩸 Macrocytic, normochromic

C. Aplastic Anemia

• Bone marrow failure → all cell lines ↓
• 🧾 Causes: Radiation, drugs (chloramphenicol), viral infections (parvovirus B19)
• Pancytopenia: ↓ RBCs, WBCs, platelets

D. Anemia of Chronic Disease

• Impaired iron utilization + reduced erythropoietin
• 🧾 Seen in: CKD, RA, TB, cancer
• 🩸 Normocytic or microcytic

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2️⃣ Anemia due to Increased RBC Destruction (Hemolytic Anemia)

A. Intrinsic (Hereditary) Hemolytic Anemias

• Sickle Cell Anemia: Abnormal HbS causes RBC sickling
• Thalassemia: Defective hemoglobin chain synthesis
• Hereditary Spherocytosis: Spherical, fragile RBCs
• G6PD Deficiency: Enzyme defect; RBCs damaged by oxidative stress

B. Extrinsic (Acquired) Hemolytic Anemias

• Autoimmune Hemolytic Anemia: Body makes antibodies against RBCs
• Mechanical destruction: Prosthetic heart valves
• Infections: Malaria, sepsis
• Toxins: Snake venom, drugs

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3️⃣ Anemia due to Blood Loss

A. Acute Blood Loss Anemia

• 🧾 Causes: Trauma, surgery, bleeding ulcers
• Normocytic, normochromic initially

B. Chronic Blood Loss Anemia

• 🧾 Causes: GI bleeding, menstruation, hookworm
• Leads to iron deficiency → microcytic, hypochromic

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🔬 II. Based on RBC Morphology (MCV and MCHC)

🩺 Type	MCV	MCHC	Examples
Microcytic Hypochromic	<80 fL	↓	Iron deficiency, Thalassemia
Normocytic Normochromic	80–100 fL	Normal	Acute blood loss, Aplastic anemia, Chronic disease
Macrocytic Normochromic	>100 fL	Normal	B12/Folate deficiency, Alcoholism

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🧬 III. Special Types of Anemia

🔷 Sickle Cell Anemia

• Genetic → HbS causes RBCs to sickle under stress
• Pain crises, organ damage
• Common in African, Indian populations

🔷 Thalassemia (α & β)

• Genetic disorder → reduced globin chain synthesis
• Microcytic anemia with target cells
• Requires frequent transfusions

🔷 Pernicious Anemia

• Autoimmune destruction of intrinsic factor → B12 deficiency
• Neurological symptoms + macrocytic anemia

🔷 Hemolytic Disease of the Newborn

• Rh⁻ mother, Rh⁺ baby → maternal antibodies destroy fetal RBCs
• Jaundice, hepatosplenomegaly in neonates

🔷 Anemia of Chronic Kidney Disease

• ↓ Erythropoietin → ↓ RBC production
• Normocytic, normochromic

🔷 Autoimmune Hemolytic Anemia

• Body produces antibodies against RBCs
• Positive Coombs test

🔷 G6PD Deficiency

• Enzyme deficiency → oxidative damage
• Triggered by infections, drugs (sulfa, anti-malarials)

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📌 Summary Table

🔢 Type	🧪 Mechanism	🧾 Example
Iron Deficiency	↓ Hb production	Poor diet, chronic blood loss
Megaloblastic	↓ DNA synthesis	B12/Folate deficiency
Aplastic	Marrow failure	Drugs, radiation
Hemolytic	↑ RBC destruction	Sickle cell, Thalassemia
Blood loss	Loss of RBCs	Trauma, GI bleeding
Chronic disease	↓ Iron use & EPO	TB, RA, CKD

🧬 Pathophysiology of All Types of Anemia

Anemia results from one or more of the following core mechanisms:

🔻 Reduced RBC production
💥 Increased RBC destruction (Hemolysis)
🩸 Blood loss

Below is a breakdown by each major type of anemia, explaining the underlying pathophysiological process.

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1️⃣ Iron Deficiency Anemia (Microcytic, Hypochromic)

🧬 Pathophysiology:

1. ↓ Iron intake, absorption, or ↑ loss →
2. ↓ Availability of iron for hemoglobin synthesis →
3. Formation of small (microcytic), pale (hypochromic) RBCs →
4. ↓ Oxygen-carrying capacity →
5. Tissue hypoxia and anemia symptoms

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2️⃣ Megaloblastic Anemia (Macrocytic, Normochromic)

👉 Includes Vitamin B12 Deficiency & Folic Acid Deficiency

🧬 Pathophysiology:

1. Deficiency of B12 or folic acid →
2. Impaired DNA synthesis in bone marrow →
3. Delayed cell division but normal cytoplasm →
4. Large, immature RBCs (megaloblasts) released →
5. Ineffective erythropoiesis → Anemia + neurological symptoms (esp. B12)

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3️⃣ Aplastic Anemia (Pancytopenia)

🧬 Pathophysiology:

1. Bone marrow failure due to drugs, radiation, viral infections →
2. Destruction or suppression of hematopoietic stem cells →
3. ↓ Production of all blood cells: RBCs, WBCs, platelets →
4. Leads to anemia, infections, and bleeding (pancytopenia)

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4️⃣ Sickle Cell Anemia (Hemolytic, Genetic)

🧬 Pathophysiology:

1. Mutation in β-globin gene → formation of abnormal HbS →
2. Under low O₂, HbS polymerizes → RBCs become sickle-shaped →
3. Rigid, sticky RBCs block vessels → vaso-occlusion → ischemia, pain
4. Sickle cells are fragile → increased hemolysis → chronic anemia

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5️⃣ Thalassemia (Microcytic, Genetic)

🧬 Pathophysiology:

1. Genetic defect → reduced or absent synthesis of α or β globin chains →
2. Unbalanced globin chains → damage RBC precursors →
3. Ineffective erythropoiesis + increased hemolysis in spleen →
4. Chronic anemia, compensatory bone marrow hyperplasia

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6️⃣ Autoimmune Hemolytic Anemia (AIHA)

🧬 Pathophysiology:

1. Immune system produces autoantibodies against patient’s own RBCs →
2. Antibody-coated RBCs destroyed by spleen or complement system →
3. ↑ Hemolysis → ↑ bilirubin → jaundice + anemia symptoms

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7️⃣ G6PD Deficiency (Hemolytic, Enzyme defect)

🧬 Pathophysiology:

1. Inherited enzyme defect (G6PD) → ↓ protection from oxidative stress →
2. Trigger (drug, infection, fava beans) → oxidative damage to RBCs →
3. Formation of Heinz bodies → cell rupture (hemolysis) → anemia

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8️⃣ Sideroblastic Anemia

🧬 Pathophysiology:

1. Defective heme synthesis → iron accumulates in mitochondria of RBC precursors
2. Formation of ringed sideroblasts in bone marrow →
3. Ineffective erythropoiesis and anemia

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9️⃣ Anemia of Chronic Disease (Normocytic or Microcytic)

🧬 Pathophysiology:

1. Chronic inflammation → ↑ hepcidin (liver hormone) →
2. ↓ Iron absorption & release from stores → functional iron deficiency
3. Cytokines suppress EPO production & marrow response →
4. Mild anemia with normal or increased ferritin

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🔟 Hemolytic Disease of the Newborn (HDN)

🧬 Pathophysiology:

1. Rh⁻ mother exposed to Rh⁺ fetal blood → makes anti-Rh antibodies
2. In next Rh⁺ pregnancy, maternal antibodies cross placenta →
3. Destruction of fetal RBCs → hemolysis, anemia, jaundice, hydrops

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1️⃣1️⃣ Post-Hemorrhagic Anemia (Acute/Chronic Blood Loss)

🧬 Pathophysiology:

🔹 Acute loss:

• Sudden hemorrhage → ↓ blood volume → hypovolemic shock
• Compensatory response: ↑ HR, vasoconstriction, fluid shift
• After 24–48 hrs: ↓ RBCs and Hb → normocytic anemia

🔹 Chronic loss:

• Gradual loss (e.g., GI bleed) → depletion of iron stores
• Leads to iron deficiency anemia (microcytic, hypochromic)

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1️⃣2️⃣ Anemia in Chronic Kidney Disease (CKD)

🧬 Pathophysiology:

1. Damaged kidneys → ↓ production of erythropoietin (EPO)
2. EPO deficiency → ↓ stimulation of bone marrow →
3. ↓ RBC production → normocytic, normochromic anemia

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🧠 Summary Chart: Mechanisms of Anemia

🔢 Type	🧬 Pathophysiology
Iron Deficiency	↓ Hb synthesis due to iron lack
Megaloblastic	Impaired DNA synthesis (B12/folate)
Aplastic	Marrow failure, pancytopenia
Hemolytic	↑ RBC destruction (intrinsic or extrinsic)
Post-hemorrhagic	RBC loss from bleeding
Sickle Cell	HbS polymerization → sickling & hemolysis
Thalassemia	Globin chain imbalance → ineffective erythropoiesis
CKD-related	↓ EPO production by kidneys
AIHA	Autoantibodies destroy RBCs
G6PD Deficiency	Oxidative damage → hemolysis
Chronic Disease	Inflammation → iron sequestration + ↓ EPO

🩸 Anemia – Signs & Symptoms and Diagnosis

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🧠 I. General Signs & Symptoms of Anemia

(All types of anemia share some common features due to reduced oxygen-carrying capacity of the blood)

🔍 System	⚠️ Clinical Signs & Symptoms
General	🔻 Fatigue, weakness, malaise, lethargy
Skin	Pallor (pale skin, mucosa, nail beds), cold intolerance
Cardiovascular	Palpitations, tachycardia, systolic murmur, hypotension
Respiratory	Dyspnea on exertion, rapid breathing
Neurological	Headache, dizziness, irritability, fainting
GI	Loss of appetite, nausea, constipation (esp. in iron deficiency)
Musculoskeletal	Muscle weakness, leg cramps
Reproductive (♀)	Menstrual irregularities, menorrhagia

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🔎 II. Type-Specific Signs & Symptoms

🔻 Iron Deficiency Anemia

• Spoon-shaped nails (koilonychia)
• Glossitis (smooth, sore tongue)
• Angular cheilitis (cracks at mouth corners)
• Pica (craving for non-food items like ice/clay)

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🧠 Vitamin B12 Deficiency Anemia

• Neurological symptoms: tingling/numbness in hands and feet
• Ataxia (difficulty walking)
• Memory issues or confusion
• Beefy red tongue

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🍃 Folic Acid Deficiency

• Similar to B12 anemia, but no neurological symptoms
• Mouth ulcers

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💥 Hemolytic Anemia

• Jaundice (due to breakdown of RBCs)
• Dark-colored urine
• Splenomegaly
• Fatigue, pallor

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🧬 Sickle Cell Anemia

• Severe pain episodes (sickle cell crisis)
• Swelling in hands and feet (dactylitis)
• Chronic anemia, delayed growth
• Frequent infections

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🧬 Thalassemia

• Bone deformities (facial, skull)
• Growth retardation
• Splenomegaly
• Bronze or pale skin

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🧫 Aplastic Anemia

• Pancytopenia signs:
  🔻 RBCs → fatigue
  🔻 WBCs → frequent infections
  🔻 Platelets → bleeding, petechiae

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🚨 Post-Hemorrhagic Anemia

• Acute: Signs of hypovolemic shock (cold, clammy skin, ↓ BP, ↑ HR)
• Chronic: Slow onset of fatigue and pallor

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🌿 Anemia of Chronic Disease

• Mild, slowly progressive fatigue
• Usually coexists with symptoms of underlying disease (e.g., TB, RA)

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🧪 III. Diagnostic Investigations

🧪 Test	🔍 Purpose
CBC (Complete Blood Count)	↓ Hb, ↓ Hct, MCV/MCH values help identify type (micro/normo/macrocytic)
Peripheral Blood Smear	Shape, size, color of RBCs (e.g., sickle cells, target cells, blasts)
Reticulocyte Count	Indicates bone marrow response (↑ in hemolysis, ↓ in aplastic anemia)
Serum Iron, Ferritin, TIBC	Evaluate iron deficiency or overload
Vitamin B12 & Folic Acid Levels	For macrocytic/megaloblastic anemia
Bone Marrow Aspiration/Biopsy	Used in suspected leukemia, aplastic anemia, marrow infiltration
Hemoglobin Electrophoresis	Detects abnormal hemoglobin types (HbS in sickle cell, HbF in thalassemia)
Coombs Test	Detects antibodies against RBCs (autoimmune hemolytic anemia)
LDH, Bilirubin (indirect), Haptoglobin	Hemolysis markers
Stool Occult Blood Test	Identify hidden GI bleeding (common in chronic iron-deficiency anemia)
Renal Function Test	To check for anemia of chronic kidney disease
ESR, CRP	Evaluate inflammation in chronic disease anemia

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📌 Diagnostic Clues by Type

🩸 Anemia Type	🔬 Key Diagnostic Findings
Iron Deficiency	↓ Hb, ↓ MCV, ↓ MCH, ↓ serum ferritin, ↑ TIBC
Megaloblastic	↑ MCV, hypersegmented neutrophils, ↓ B12/Folate
Sickle Cell	HbS on electrophoresis, sickle cells in smear
Thalassemia	↓ MCV, target cells, ↑ HbF/HbA2 on electrophoresis
Aplastic	Pancytopenia in CBC, hypocellular marrow
Hemolytic	↑ reticulocytes, ↑ LDH, ↑ indirect bilirubin, ↓ haptoglobin
CKD-related	↓ Hb, normocytic cells, ↓ erythropoietin
Post-hemorrhagic	↓ Hb/Hct, normal MCV early, signs of shock in acute loss

🩸 Anemia – Medical and Surgical Management

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💊 I. Medical Management (Type-Specific)

🔻 1. Iron Deficiency Anemia

A. Iron Replacement Therapy

• Oral Iron supplements:
  ➤ Ferrous sulfate, Ferrous gluconate
  ➤ Dose: 100–200 mg elemental iron/day
  ➤ Administer with Vitamin C to improve absorption
  ➤ Side effects: constipation, black stools, nausea
• Parenteral Iron (IM/IV) (for malabsorption or intolerance):
  ➤ Iron sucrose, Ferric carboxymaltose

B. Dietary Advice

• Iron-rich foods: red meat, green leafy vegetables, jaggery, dates, legumes
• Avoid tea/coffee with meals (↓ absorption)

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🧠 2. Vitamin B12 Deficiency Anemia

A. Vitamin B12 Replacement

• IM injections of cyanocobalamin or hydroxocobalamin
  ➤ Initial: 1000 mcg IM daily × 7 days, then weekly, then monthly
• Oral B12 supplements (for mild cases)

B. Dietary Management

• Include animal products: meat, eggs, milk
• For vegetarians: consider lifelong oral/IM supplementation

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🍃 3. Folic Acid Deficiency Anemia

• Folic acid supplements: 5 mg/day PO
• Dietary sources: green vegetables, citrus fruits, legumes, liver

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💥 4. Hemolytic Anemias

A. General Support

• Folic acid to support new RBC formation
• Hydration to prevent hemoglobin nephropathy
• Oxygen therapy (if hypoxic)

B. Autoimmune Hemolytic Anemia

• Corticosteroids (e.g., Prednisolone)
• Immunosuppressants (if unresponsive)
• Plasmapheresis in severe cases

C. G6PD Deficiency

• Avoid triggering drugs (sulfa, anti-malarials, fava beans)
• Treat infections promptly
• Blood transfusions in crisis

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🧬 5. Sickle Cell Anemia

• Hydroxyurea to reduce sickling & painful crises
• Folic acid supplementation
• Pain management: NSAIDs, opioids during crises
• IV fluids, O₂ therapy during vaso-occlusive crisis
• Antibiotics for infections
• Routine vaccinations

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🧬 6. Thalassemia (Major)

• Regular blood transfusions to maintain Hb >9–10 g/dL
• Iron chelation therapy (e.g., Deferoxamine, Deferasirox)
• Folic acid supplementation
• Avoid iron supplements

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🧫 7. Aplastic Anemia

• Immunosuppressive therapy: ATG (antithymocyte globulin), Cyclosporine
• Growth factors: G-CSF, EPO
• Transfusions: PRBCs, platelets
• Antibiotics/antifungals: for infections

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🌿 8. Anemia of Chronic Disease

• Treat underlying cause (e.g., TB, RA, CKD)
• Erythropoiesis-Stimulating Agents (ESAs) in CKD
• Iron supplementation (if iron-restricted)

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🚨 9. Acute Post-Hemorrhagic Anemia

• IV Fluids: to manage shock
• Blood transfusion: if Hb <7 or ongoing bleed
• Monitor vital signs, urine output, CVP

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🩻 II. Surgical Management (When Indicated)

While anemia is primarily treated medically, surgical interventions may be necessary in select cases:

🧬 1. Splenectomy

🔹 Indication:

• Hereditary spherocytosis
• Thalassemia with hypersplenism
• Autoimmune hemolytic anemia unresponsive to drugs
  🔹 Purpose:
• Reduce RBC destruction
• Improve anemia control

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🩸 2. Bone Marrow / Stem Cell Transplant

🔹 Indication:

• Aplastic anemia (young patients)
• Thalassemia major
• Sickle cell anemia (in select cases)
  🔹 Purpose:
• Replace defective bone marrow with healthy donor cells
  🔹 Types:
• Allogeneic (from donor)
• Autologous (from patient)

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🧫 3. Surgery to Treat Cause of Bleeding

🔹 Examples:

• Peptic ulcer repair (if GI bleeding)
• Hysterectomy (for fibroid-induced bleeding)
• Polypectomy (colon polyps causing occult bleeding)
  🔹 Outcome:
• Stop blood loss and correct anemia cause

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🚨 4. Emergency Surgery in Acute Hemorrhage

🔹 Examples:

• Trauma-related bleeding
• Ruptured ectopic pregnancy
• Splenic rupture
  🔹 Support:
• Blood transfusion and volume resuscitation required pre/post-op

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✅ Summary Table: Management Overview

🔢 Type	💊 Medical Management	🏥 Surgical Option
Iron Deficiency	Oral/IV Iron	Treat source of bleeding
B12/Folate Def.	B12/folate therapy	None
Aplastic	Immunosuppressants	Bone marrow transplant
Hemolytic	Steroids, Folic acid	Splenectomy
Sickle Cell	Hydroxyurea, transfusion	Bone marrow transplant
Thalassemia	Transfusions, chelation	Splenectomy, BMT
Post-Hemorrhagic	Transfusion, fluids	Emergency surgery
CKD-related	EPO, iron	Dialysis (supportive)

🩺 Nursing Management of Anemia

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🧠 I. General Nursing Objectives

• Improve tissue oxygenation
• Prevent complications (e.g., infections, bleeding)
• Promote nutritional intake
• Manage fatigue and activity intolerance
• Educate the patient and family about disease and care

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📋 II. General Nursing Interventions

1️⃣ Assessment

• Monitor vital signs: 🩺 HR, BP, RR, temperature
• Assess for signs of:
  • Pallor (skin, mucous membranes, conjunctiva)
  • Fatigue, dizziness, weakness
  • Shortness of breath, tachycardia
  • Bleeding (gums, stool, bruising)
• Monitor lab values: CBC, Hb, Hct, MCV, serum iron, B12, folate

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2️⃣ Improve Oxygenation and Tissue Perfusion

• Administer oxygen therapy if SpO₂ < 90%
• Elevate head of bed to ease breathing
• Monitor for signs of hypoxia (confusion, cyanosis)
• Encourage rest and minimize exertion
• Provide blood transfusion if ordered (monitor for reactions)

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3️⃣ Manage Fatigue

• Prioritize activities; allow rest periods
• Assist with ADLs when needed
• Educate on energy-conserving techniques
• Gradually increase physical activity as tolerated

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4️⃣ Nutritional Management

• Encourage high-iron foods: meats, leafy greens, legumes, dates
• For B12 deficiency: include meat, eggs, dairy
• For folate deficiency: green vegetables, citrus, whole grains
• Administer iron, B12, or folic acid supplements as prescribed
• Educate on avoiding tea/coffee with meals (inhibits iron absorption)

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5️⃣ Infection and Bleeding Prevention (esp. in aplastic or hemolytic anemia)

• Maintain strict hand hygiene
• Monitor temperature regularly (fever → early infection)
• Use soft toothbrush, avoid invasive procedures if thrombocytopenic
• Watch for petechiae, hematuria, bleeding gums
• Apply pressure to injection sites

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6️⃣ Psychosocial and Emotional Support

• Provide reassurance and emotional support
• Address fears or concerns related to chronic illness or transfusions
• Encourage patient involvement in care planning
• Refer to support groups or counseling if needed

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7️⃣ Health Education

• Importance of medication compliance (iron/B12/folate)
• Dietary counseling tailored to anemia type
• Educate about signs of worsening anemia or complications
• Teach safe medication storage and iron toxicity risks (esp. in children)
• Explain follow-up lab monitoring needs

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🔍 III. Nursing Interventions Based on Anemia Type

🩸 Type of Anemia	👩‍⚕️ Specific Nursing Focus
Iron Deficiency	Administer oral/parenteral iron, prevent GI side effects, monitor ferritin & Hb
Vitamin B12 Deficiency	Monitor neurological status, give IM B12, teach lifelong therapy if pernicious anemia
Folic Acid Deficiency	Provide folate-rich diet, administer supplements
Aplastic Anemia	Monitor for infection/bleeding, apply protective isolation if needed, support transfusions
Sickle Cell Anemia	Pain management, hydration, oxygen therapy, infection control, crisis prevention
Thalassemia Major	Regular transfusion care, monitor iron overload, support chelation therapy
Hemolytic Anemia	Monitor jaundice, urine color, provide corticosteroids or immune therapy education
Post-Hemorrhagic Anemia	Monitor for shock, support IV fluids and transfusions, prepare for emergency interventions
Chronic Disease Anemia	Support disease management, administer EPO, iron (if needed), manage fatigue

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🧾 IV. Sample Nursing Diagnoses

• Activity intolerance related to decreased oxygen supply
• Risk for bleeding related to thrombocytopenia
• Imbalanced nutrition: less than body requirements
• Fatigue related to decreased hemoglobin
• Risk for infection related to immunosuppression
• Knowledge deficit related to disease and self-care

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✅ V. Evaluation Criteria

• Improved Hb and RBC levels
• Decreased fatigue; improved activity tolerance
• No signs of bleeding or infection
• Patient follows dietary/medication plan
• Patient demonstrates understanding of condition

⚠️ Complications of Anemia

Anemia, if untreated or severe, can lead to various complications depending on its type, severity, and underlying cause.

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🩸 I. General Complications of Anemia

⚠️ Complication	🧠 Explanation
Hypoxia	↓ Hemoglobin → ↓ Oxygen to tissues → organ dysfunction
Cardiac strain / Heart failure	Heart compensates by pumping faster → leads to left ventricular hypertrophy or heart failure
Tachycardia & Palpitations	Compensation for low oxygen levels
Fatigue and reduced productivity	Due to low energy supply to tissues
Shortness of breath	Poor oxygenation of blood
Pallor & Dizziness → Falls/injury risk	Especially in elderly
Cognitive dysfunction	Impaired memory, confusion in chronic anemia
Menstrual irregularities or infertility	In women with long-term anemia
Poor growth and development in children	Especially in iron-deficiency and thalassemia

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💉 II. Type-Specific Complications

🔻 Iron Deficiency Anemia

• Pica (eating non-food items)
• Impaired cognitive function in children
• Restless leg syndrome

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🧠 Vitamin B12 Deficiency Anemia

• Irreversible neurological damage: tingling, numbness, ataxia
• Cognitive impairment, memory loss
• Depression or psychosis in extreme cases

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💥 Hemolytic Anemia

• Jaundice due to excess bilirubin
• Gallstones from chronic hemolysis
• Splenomegaly
• Risk of aplastic crisis (especially in sickle cell or hereditary spherocytosis)

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🧬 Sickle Cell Anemia

• Pain crises (vaso-occlusive)
• Stroke
• Acute chest syndrome
• Leg ulcers
• Organ damage: kidneys, liver, lungs, eyes
• Frequent infections

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🧬 Thalassemia

• Iron overload due to frequent transfusions → leads to liver, heart, pancreas damage
• Bone deformities
• Delayed puberty
• Growth retardation

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🧫 Aplastic Anemia

• Severe infections (due to leukopenia)
• Uncontrolled bleeding (due to thrombocytopenia)
• High mortality if untreated

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🧪 Post-Hemorrhagic Anemia

• Hypovolemic shock
• Organ failure if severe/untreated

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📌 III. Key Points for Nursing Students & Clinical Practice

✅ Anemia is a symptom, not a disease – always assess the underlying cause.

✅ Early signs include fatigue, pallor, and weakness – watch for subtle signs in elderly and children.

✅ Always monitor lab values: Hb, Hct, RBC, MCV, iron profile, vitamin levels.

✅ Provide nutritional counseling based on anemia type – iron, B12, folate, protein.

✅ Teach correct iron therapy: empty stomach, with Vitamin C, avoid tea/coffee.

✅ Transfusion reactions must be closely monitored in patients receiving blood.

✅ Infection control is vital in patients with aplastic or sickle cell anemia.

✅ Watch for neurological symptoms in B12 deficiency – early detection prevents permanent damage.

✅ Educate patients on medication compliance, follow-up testing, and lifestyle modifications.

✅ Multidisciplinary approach (dietician, hematologist, counselor) is often needed in chronic or genetic anemia.

🩸 POLYCYTHEMIA

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📘 Definition

Polycythemia is a blood disorder characterized by an abnormally increased number of red blood cells (RBCs) in the bloodstream, which leads to increased blood viscosity and volume, potentially causing circulatory issues and thrombotic complications.

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❓ Causes

Polycythemia may arise due to primary, secondary, or relative mechanisms:

🔹 Primary Cause:

• Bone marrow disorder leading to uncontrolled RBC production
• Example: Polycythemia Vera (PV) – a chronic myeloproliferative neoplasm

🔹 Secondary Cause:

• Overproduction of erythropoietin (EPO) due to:
  • Chronic hypoxia (COPD, sleep apnea, heart disease)
  • High altitudes
  • EPO-secreting tumors (renal or hepatic cancers)

🔹 Relative Cause:

• Hemoconcentration due to reduced plasma volume (not true increase in RBCs)
  • Seen in dehydration, vomiting, burns, stress

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🔠 Types of Polycythemia

Type	Cause	Notes
1. Primary Polycythemia (Polycythemia Vera)	Myeloproliferative bone marrow disorder	JAK2 mutation positive
2. Secondary Polycythemia	↑ EPO due to hypoxia or tumors	Reversible if underlying cause is treated
3. Relative Polycythemia	↓ Plasma volume	Common in dehydration or stress

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🧬 Pathophysiology

A. Polycythemia Vera (PV)

1. JAK2 gene mutation → activates signaling without EPO
2. Bone marrow produces excessive RBCs, WBCs, and platelets
3. ↑ Blood viscosity → ↓ tissue perfusion
4. Risk of thrombosis, hemorrhage, splenomegaly

B. Secondary Polycythemia

1. Chronic hypoxia or tumor → ↑ EPO secretion
2. EPO stimulates bone marrow → ↑ RBC production
3. Increased oxygen delivery temporarily helps but raises blood viscosity

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⚠️ Signs and Symptoms

System	Symptoms
General	Ruddy complexion, flushing, headache, fatigue, dizziness
Neurological	Blurred vision, tinnitus, confusion, lightheadedness
Cardiovascular	Hypertension, chest pain, palpitations
Respiratory	Shortness of breath, sleep apnea
Vascular	Itching (especially after hot shower), thrombosis, erythromelalgia (burning in hands/feet)
GI/Spleen	Splenomegaly, early satiety, abdominal discomfort
Bleeding	Nosebleeds, gum bleeding, GI bleeding in later stages

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🔬 Diagnosis

Test	Findings
CBC	↑ Hb, Hct, RBC count
Erythropoietin level	↓ in PV; ↑ in secondary polycythemia
JAK2 mutation test	Positive in most PV cases
Oxygen saturation (SpO₂)	↓ in secondary due to hypoxia
ABG (Arterial Blood Gas)	Hypoxia in secondary type
Bone marrow biopsy	Hypercellular marrow in PV
Chest X-ray/CT scan	Rule out pulmonary or renal causes
Ultrasound abdomen	Check for renal tumors or splenomegaly

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💊 Medical Management

For Polycythemia Vera (PV):

• Phlebotomy: 300–500 mL blood removed periodically to reduce hematocrit
• Hydroxyurea: suppresses bone marrow activity
• Ruxolitinib: JAK2 inhibitor (targeted therapy)
• Low-dose aspirin: prevents thrombosis
• Allopurinol: controls hyperuricemia (due to cell breakdown)
• Antihistamines: relieve pruritus

For Secondary Polycythemia:

• Treat underlying cause (e.g., oxygen for COPD, surgery for tumors)
• Avoid smoking or high altitudes
• Controlled oxygen therapy
• Phlebotomy if symptoms persist

For Relative Polycythemia:

• Rehydration and fluid balance correction
• Treat dehydration causes (vomiting, diarrhea, burns)

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🏥 Surgical Management

Surgery is not a primary treatment for polycythemia but may be necessary in cases of:

1. Renal or hepatic tumor removal

• If EPO-producing tumors cause secondary polycythemia

2. Splenectomy

• In cases of severe splenomegaly causing pain, infarction, or rupture

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👩‍⚕️ Nursing Management

✅ Nursing Assessment

• Monitor vitals (especially BP, HR)
• Assess for signs of thromboembolism: redness, swelling, pain in limbs
• Observe bleeding from gums, nose, injection sites
• Monitor lab values: CBC, Hct, EPO, JAK2

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✅ Nursing Interventions

• Promote hydration to reduce blood viscosity
• Administer medications as ordered (hydroxyurea, aspirin)
• Assist during phlebotomy, monitor for dizziness or hypotension
• Apply pressure after injections/venipuncture to prevent bleeding
• Provide oxygen therapy if hypoxic
• Educate patient:
  • Avoid high altitudes, smoking
  • Avoid iron supplements unless directed
  • Maintain fluid intake
  • Recognize early signs of clotting or bleeding

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✅ Health Education

• Lifestyle changes: quit smoking, avoid dehydration
• Importance of medication and follow-up compliance
• Avoid vigorous exercise if at risk for thrombosis
• Use loose-fitting clothing (avoid circulatory restriction)

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⚠️ Complications of Polycythemia

🔥 Complication	💥 Description
Thrombosis	Stroke, myocardial infarction, DVT due to hyperviscosity
Bleeding	Due to dysfunctional platelets despite high count
Splenic infarction or rupture	From enlarged spleen
Gout	From excess uric acid
Progression to leukemia	Especially in Polycythemia Vera (rare)

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📌 Key Points to Remember

✅ Polycythemia = ↑ RBCs, ↑ blood viscosity → ↑ risk of thrombosis
✅ Polycythemia Vera is caused by JAK2 mutation
✅ Phlebotomy is the first-line treatment for PV
✅ EPO levels help differentiate primary (low EPO) vs. secondary (high EPO)
✅ Always assess for clotting and bleeding signs
✅ Avoid iron unless strictly indicated
✅ Patient education and regular monitoring are critical for long-term management

🩸 Bleeding Disorders

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📘 Definition

Bleeding disorders are a group of medical conditions that result in abnormal bleeding due to defects in the blood clotting (coagulation) system, including problems with:

• Platelets
• Clotting factors
• Blood vessels

These disorders may cause prolonged, spontaneous, or excessive bleeding after injury, surgery, or even without any apparent trauma.

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❓ Causes of Bleeding Disorders

Bleeding disorders can be congenital (inherited) or acquired (developed later in life).

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🔹 A. Inherited (Congenital) Causes

Disorder	Description
Hemophilia A	Deficiency of clotting factor VIII
Hemophilia B (Christmas disease)	Deficiency of factor IX
Von Willebrand Disease	Deficiency or dysfunction of von Willebrand factor (vWF), affecting platelet adhesion and factor VIII
Rare factor deficiencies	Factor I, II, V, VII, X, XI, XIII deficiencies (very rare)
Platelet function disorders	Example: Glanzmann’s thrombasthenia, Bernard-Soulier syndrome

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🔹 B. Acquired Causes

Cause	Description
Liver disease	Liver produces most clotting factors; damage leads to deficiency
Vitamin K deficiency	Needed for synthesis of factors II, VII, IX, X
Disseminated Intravascular Coagulation (DIC)	Widespread clotting uses up clotting factors and platelets, leading to bleeding
Autoimmune diseases	e.g., Immune thrombocytopenic purpura (ITP) – immune destruction of platelets
Medications	Anticoagulants (e.g., warfarin, heparin), antiplatelets (aspirin, clopidogrel)
Chemotherapy or radiation	Bone marrow suppression → ↓ platelet production
Bone marrow disorders	Aplastic anemia, leukemia → ↓ platelet or factor production
Massive transfusion syndrome	Dilutional coagulopathy due to large volume transfusions without clotting factors

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🔹 C. Vascular Causes (Rare)

• Ehlers-Danlos syndrome, Hereditary hemorrhagic telangiectasia
• Fragile blood vessels that bleed easily

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🧠 Summary of Categories

🔢 Category	💥 Example Causes
Platelet Disorders	ITP, thrombocytopenia, drug-induced
Clotting Factor Deficiency	Hemophilia A/B, liver disease, Vitamin K deficiency
Vascular Defects	Ehlers-Danlos, senile purpura
Mixed	DIC, severe liver failure

🩸 Types of Bleeding Disorders

Bleeding disorders are broadly classified into:

✅ 1. Platelet Disorders

✅ 2. Clotting Factor Deficiencies

✅ 3. Vascular Disorders

✅ 4. Mixed Disorders (Multiple Mechanisms)

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1️⃣ Platelet Disorders (Quantitative or Qualitative)

These affect the number or function of platelets, which are essential for clot formation.

🔹 A. Quantitative Platelet Disorders (Thrombocytopenia)

Decreased platelet count

Disorder	Description
Immune Thrombocytopenic Purpura (ITP)	Autoimmune destruction of platelets
Thrombotic Thrombocytopenic Purpura (TTP)	Microthrombi consume platelets → low count
Drug-induced thrombocytopenia	Heparin-induced thrombocytopenia (HIT), chemotherapy
Bone marrow suppression	Aplastic anemia, leukemia, radiation
Infections	Dengue, HIV, sepsis
Hypersplenism	Spleen traps excessive platelets

🔹 B. Qualitative Platelet Disorders (Dysfunction)

Platelets are present but don’t work properly

Disorder	Description
Glanzmann’s Thrombasthenia	Defect in platelet aggregation
Bernard-Soulier Syndrome	Defect in platelet adhesion
Uremia-related platelet dysfunction	Seen in chronic kidney disease
Drug-induced	Aspirin, NSAIDs interfere with platelet function

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2️⃣ Clotting Factor Deficiencies

🔹 A. Inherited Clotting Factor Deficiencies

Disorder	Deficient Factor	Notes
Hemophilia A	Factor VIII	X-linked recessive
Hemophilia B (Christmas disease)	Factor IX	Similar to A, less common
Hemophilia C	Factor XI	Autosomal recessive
Von Willebrand Disease (vWD)	vWF (also affects Factor VIII)	Most common inherited bleeding disorder
Rare Factor Deficiencies	Factors I (Fibrinogen), II, V, VII, X, XIII	Extremely rare; variable severity

🔹 B. Acquired Clotting Factor Deficiencies

Disorder	Cause
Vitamin K Deficiency	Needed for factors II, VII, IX, X
Liver Disease	Liver synthesizes most clotting factors
Anticoagulant Overdose	Warfarin, heparin
Massive Transfusion Syndrome	Dilution of clotting factors
Disseminated Intravascular Coagulation (DIC)	Clotting and bleeding occur simultaneously

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3️⃣ Vascular Disorders (Bleeding due to fragile blood vessels)

Disorder	Description
Hereditary Hemorrhagic Telangiectasia	Abnormal blood vessels → easy bleeding
Ehlers-Danlos Syndrome	Connective tissue disorder → fragile vessels
Senile Purpura	Age-related capillary fragility in elderly
Infectious Vasculitis	Seen in meningococcemia, dengue

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4️⃣ Mixed Disorders (Multiple Defects)

Disorder	Description
Disseminated Intravascular Coagulation (DIC)	Widespread clotting uses up platelets and factors → bleeding
Liver Failure	Affects platelets, clotting factors, and fibrinolysis
Uremia	Affects platelet function and clotting factor activity
Systemic Lupus Erythematosus (SLE)	Autoimmune platelet destruction + clotting abnormalities
Leukemia	Affects bone marrow production of both platelets and factors

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📌 Summary Chart: Classification of Bleeding Disorders

🔢 Category	🧬 Example Disorders
Platelet Disorders	ITP, TTP, Glanzmann’s, Aspirin effect
Clotting Factor Disorders	Hemophilia A/B, vWD, Vit K deficiency
Vascular Disorders	Ehlers-Danlos, Senile purpura
Mixed Disorders	DIC, liver failure, leukemia

🧬 Pathophysiology of Bleeding Disorders (All Types)

Bleeding disorders occur due to defects in three main areas:

1. 🧪 Platelet count or function
2. 🧬 Clotting factor production or activity
3. 🧫 Vessel wall integrity

Each type leads to impaired hemostasis (blood clot formation), resulting in prolonged, excessive, or spontaneous bleeding.

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1️⃣ Platelet Disorders

🔹 A. Thrombocytopenia (Low Platelet Count)

Pathophysiology:

1. ↓ Platelet production in bone marrow
   → e.g., leukemia, aplastic anemia, chemo
2. ↑ Platelet destruction
   → immune-mediated (ITP), DIC
3. ↑ Platelet sequestration
   → in hypersplenism or splenomegaly
4. Platelet count <150,000/μL → impaired formation of platelet plug
5. Results in petechiae, purpura, mucosal bleeding

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🔹 B. Platelet Dysfunction (Qualitative Disorders)

Pathophysiology:

1. Platelets are present but can’t adhere or aggregate properly
2. Caused by inherited defects (e.g., Glanzmann’s thrombasthenia, Bernard-Soulier)
   or acquired (e.g., aspirin, NSAIDs)
3. Defective primary hemostasis → poor clot initiation → mucocutaneous bleeding

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2️⃣ Clotting Factor Deficiencies

🔹 A. Hemophilia A & B

Pathophysiology:

1. X-linked recessive mutation → deficiency of Factor VIII (A) or IX (B)
2. Normal platelet plug forms, but secondary hemostasis fails
3. Fibrin mesh not formed → unstable clot → deep bleeding into joints (hemarthrosis), muscles

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🔹 B. Von Willebrand Disease (vWD)

Pathophysiology:

1. ↓ or defective vWF, which:
   • Helps platelets adhere to damaged vessels
   • Stabilizes Factor VIII
2. Leads to both platelet dysfunction and Factor VIII deficiency
3. Results in mucosal bleeding, heavy periods, easy bruising

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🔹 C. Vitamin K Deficiency

Pathophysiology:

1. Vitamin K is needed to activate clotting factors II, VII, IX, X
2. Deficiency (due to malabsorption, liver disease, antibiotics) → ↓ activated factors
3. Leads to poor clot formation → bleeding

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🔹 D. Liver Disease

Pathophysiology:

1. Liver produces most clotting factors and fibrinogen
2. Liver dysfunction → ↓ production of clotting factors
3. Also affects platelet function and fibrinolysis
4. Causes prolonged bleeding time and INR → spontaneous bleeding

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3️⃣ Vascular Disorders

🔹 A. Hereditary Hemorrhagic Telangiectasia, Ehlers-Danlos

Pathophysiology:

1. Structural defect in blood vessel walls
2. Capillaries become fragile → break easily even with mild trauma
3. Platelets and clotting factors may be normal, but bleeding occurs due to weak vessel integrity
4. Results in skin purpura, epistaxis, GI bleeding

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4️⃣ Mixed Disorders

🔹 A. Disseminated Intravascular Coagulation (DIC)

Pathophysiology:

1. Trigger (infection, trauma, malignancy) → widespread activation of clotting cascade
2. Microthrombi form throughout the body → uses up clotting factors and platelets
3. Leads to consumptive coagulopathy
4. Results in both thrombosis and severe bleeding
   • Bleeding from IV sites, gums, GI tract
   • Organ damage due to clots

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🔹 B. Uremic Bleeding (in CKD)

Pathophysiology:

1. Uremic toxins interfere with platelet adhesion and aggregation
2. Platelet count may be normal but function is impaired
3. Results in mucosal bleeding, ecchymosis, prolonged bleeding time

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🔹 C. Leukemia / Aplastic Anemia

Pathophysiology:

1. Bone marrow failure → ↓ production of:
   • Platelets → thrombocytopenia
   • Clotting factors (if liver is also involved)
2. Leads to severe bleeding risk

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📌 Summary Table: Pathophysiology by Type

🔢 Type	🔬 Mechanism
ITP, TTP	↓ Platelets due to immune destruction or consumption
Hemophilia A/B	↓ Factor VIII/IX → impaired secondary clotting
vWD	↓ vWF → poor platelet adhesion + ↓ Factor VIII
Vitamin K Deficiency	↓ Factors II, VII, IX, X activation
Liver Disease	↓ Clotting factor production
Vascular Disorders	Fragile vessels → bleeding despite normal clotting
DIC	Overuse of platelets and factors → clotting + bleeding
CKD (Uremia)	Platelet dysfunction from toxin buildup
Leukemia	↓ Platelets due to marrow suppression

🩸 Bleeding Disorders – Signs & Symptoms and Diagnosis

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🧠 I. Signs and Symptoms of Bleeding Disorders

Signs and symptoms vary based on the type of bleeding disorder (platelet-related, clotting factor deficiency, or vascular defect). However, prolonged, excessive, or spontaneous bleeding is the hallmark of all types.

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🔹 A. General Symptoms (Common to Most Types)

🔍 Symptom	🧠 Clinical Relevance
Easy bruising (Ecchymosis)	Minor trauma causes large bruises
Petechiae	Pinpoint red spots (seen in platelet disorders)
Prolonged bleeding after injury or surgery	Impaired hemostasis
Excessive menstrual bleeding (Menorrhagia)	Common in vWD and platelet disorders
Bleeding gums or from nose (Epistaxis)	Mucosal bleeding common in platelet disorders
Blood in stool (Melena) or urine (Hematuria)	GI or urinary tract bleeding
Fatigue, pallor, dizziness	From chronic blood loss (leading to anemia)

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🔹 B. Type-Specific Symptoms

🩸 Type	⚠️ Key Signs & Symptoms
Hemophilia A/B	Deep tissue bleeding, hemarthrosis (joint swelling, pain), muscle hematomas, post-operative bleeding
Von Willebrand Disease	Nosebleeds, heavy periods, gum bleeding, prolonged bleeding time, easy bruising
ITP (Immune Thrombocytopenia)	Petechiae, purpura, mucosal bleeding, sudden onset in children after viral illness
TTP	Bleeding + neurological symptoms (confusion, headache), renal failure
DIC (Disseminated Intravascular Coagulation)	Bleeding from multiple sites (IV lines, gums), shock, bruising, organ failure
Liver Disease	Bleeding, jaundice, ascites, splenomegaly
Vitamin K Deficiency	Easy bruising, GI bleeding, prolonged PT/INR
Vascular Disorders (e.g., Ehlers-Danlos)	Fragile skin, joint hypermobility, spontaneous bleeding without trauma
Uremic Bleeding (CKD)	Mucosal bleeding, prolonged bleeding time, platelet dysfunction

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🔬 II. Diagnostic Investigations

A combination of clinical history, physical examination, and lab tests is essential for diagnosis.

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🔹 A. Basic Blood Tests

🧪 Test	🔬 Purpose
CBC (Complete Blood Count)	↓ Platelets (in thrombocytopenia), check for anemia due to chronic bleeding
Peripheral Blood Smear	To assess platelet morphology, abnormal cells (e.g., blasts in leukemia)
Reticulocyte count	Elevated in response to blood loss
Bleeding Time (BT)	Prolonged in platelet disorders, vWD
Clotting Time (CT)	Prolonged in hemophilia or factor deficiencies

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🔹 B. Coagulation Profile

Test	Normal Range	Interpretation
PT (Prothrombin Time)	11–13.5 sec	🔼 in Vitamin K deficiency, liver disease, warfarin use
INR (International Normalized Ratio)	0.8–1.1 (normal)	🔼 bleeding risk if >1.5
aPTT (Activated Partial Thromboplastin Time)	25–35 sec	🔼 in Hemophilia A/B, vWD, heparin therapy
Thrombin Time (TT)	14–21 sec	Prolonged in fibrinogen abnormalities
D-Dimer	<500 ng/mL	🔼 in DIC, thrombosis, pulmonary embolism

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🔹 C. Specialized Tests

Test	Purpose
Factor Assays (VIII, IX, etc.)	Confirms type/severity of hemophilia or rare deficiencies
Von Willebrand Factor Antigen & Activity	Confirms von Willebrand Disease
Platelet Aggregation Test	For qualitative platelet defects (e.g., Glanzmann’s)
Mixing Studies (PTT correction)	Differentiates factor deficiency vs. inhibitor presence
Bone Marrow Aspiration	In leukemia, aplastic anemia, unexplained thrombocytopenia
Liver Function Test (LFT)	Liver disease causing clotting factor deficiency
Renal Function Test (RFT)	Uremic bleeding evaluation
Genetic Testing	For inherited disorders (e.g., Hemophilia gene, vWF mutations)

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🔹 D. Imaging (if internal bleeding suspected)

• Ultrasound/CT scan – for abdominal bleeding or hematomas
• MRI joints – in hemophilia with joint involvement

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📌 Summary Table: Diagnostic Clues

Disorder	Diagnostic Clues
Hemophilia A/B	↑ aPTT, normal PT, ↓ Factor VIII/IX
vWD	↑ BT, ↑ aPTT, ↓ vWF antigen/activity
ITP	↓ Platelet count, normal PT/aPTT, clinical petechiae
DIC	↓ Platelets, ↑ PT/aPTT/INR, ↑ D-dimer, schistocytes on smear
Vitamin K deficiency	↑ PT/INR, ↓ Vitamin K levels
Liver disease	↑ PT, aPTT, ↓ clotting factors, abnormal LFTs

💊 Medical Management of Bleeding Disorders (All Types)

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1️⃣ Platelet Disorders

A. Immune Thrombocytopenic Purpura (ITP)

🔹 Goals:

• Increase platelet count
• Prevent serious bleeding

🔹 Management:

• Corticosteroids (e.g., Prednisolone): ↓ immune destruction of platelets
• IVIG (Intravenous Immunoglobulin): For rapid platelet increase
• Anti-D Immunoglobulin: In Rh-positive, non-splenectomized patients
• Immunosuppressants: Rituximab, Azathioprine (if refractory)
• Platelet transfusion: Only if active bleeding or <10,000/µL
• Splenectomy (if chronic/refractory)

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B. Thrombotic Thrombocytopenic Purpura (TTP)

🔹 Emergency Condition

• Plasma exchange (plasmapheresis) – removes autoantibodies
• Corticosteroids
• Rituximab for severe cases
• Avoid platelet transfusions unless life-threatening bleeding
• Caplacizumab (inhibits vWF–platelet interaction, if available)

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C. Drug-induced Thrombocytopenia

• Stop the offending drug (e.g., Heparin → consider HIT)
• Monitor platelet count
• Supportive care ± transfusion

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D. Platelet Function Disorders (e.g., Glanzmann’s, Bernard-Soulier)

• Antifibrinolytics: Tranexamic acid, Epsilon aminocaproic acid
• Recombinant factor VIIa (rFVIIa) in Glanzmann’s
• Platelet transfusions for bleeding episodes
• Avoid aspirin/NSAIDs

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2️⃣ Clotting Factor Deficiencies

A. Hemophilia A (Factor VIII Deficiency)

• Factor VIII concentrate (recombinant or plasma-derived)
• Desmopressin (DDAVP): In mild cases → releases stored factor VIII
• Antifibrinolytics for mucosal bleeding
• Avoid IM injections, NSAIDs
• Gene therapy (emerging treatment in select centers)

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B. Hemophilia B (Factor IX Deficiency)

• Factor IX concentrate
• Desmopressin not effective
• Same precautions as Hemophilia A

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C. Von Willebrand Disease (vWD)

• Desmopressin (DDAVP): Stimulates vWF and factor VIII release (Type 1 vWD)
• vWF–Factor VIII concentrate: For moderate-severe cases or surgery
• Antifibrinolytics: For dental/GI bleeding
• Estrogen therapy: In females with heavy menstruation

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D. Vitamin K Deficiency

• Vitamin K1 (phytonadione): Oral or IV (in severe bleeding)
• Fresh Frozen Plasma (FFP): Immediate clotting factor replacement if bleeding
• Avoid warfarin or monitor closely

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E. Rare Factor Deficiencies (II, V, VII, X, XI, XIII)

• Factor-specific concentrate if available
• FFP or cryoprecipitate as alternatives
• Antifibrinolytics for mucosal bleeding

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3️⃣ Mixed Disorders

A. Disseminated Intravascular Coagulation (DIC)

• Treat underlying cause (infection, trauma, obstetric issue)
• Platelet transfusion if <50,000 + bleeding
• FFP to replace clotting factors
• Cryoprecipitate for fibrinogen <100 mg/dL
• Heparin (low dose) only if thrombosis predominates
• Supportive care: fluids, oxygen, monitor organ function

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B. Liver Disease-Related Bleeding

• Vitamin K supplementation
• FFP for factor replacement
• Platelet transfusions if low
• Cryoprecipitate for low fibrinogen
• Manage portal hypertension (e.g., propranolol) if varices present

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C. Uremic Bleeding (CKD-related)

• Desmopressin (DDAVP)
• Dialysis: removes uremic toxins
• Cryoprecipitate: if bleeding severe
• Avoid NSAIDs
• Estrogens (rarely used) can reduce bleeding in uremia

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4️⃣ Vascular Disorders

A. Ehlers-Danlos Syndrome / Hereditary Hemorrhagic Telangiectasia

• No specific clotting deficiency
• Avoid trauma, surgical injury
• Laser therapy for mucosal telangiectasias
• Iron supplements for chronic blood loss
• Antifibrinolytics for recurrent nosebleeds or GI bleeding

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B. Senile Purpura

• Avoid trauma/friction
• Gentle skin care
• Vitamin C (questionable benefit)
• No curative therapy

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5️⃣ Supportive Management for All Bleeding Disorders

• Iron therapy for chronic blood loss
• Blood transfusion if significant anemia (PRBCs)
• Avoid aspirin, NSAIDs, and intramuscular injections
• Soft toothbrush, electric razors, and careful oral hygiene
• Patient and family education on emergency bleeding signs
• Vaccination (e.g., Hepatitis B) before starting factor replacement (esp. in Hemophilia)

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📌 Summary Table

🔢 Disorder	💊 Key Drugs
Hemophilia A	Factor VIII, DDAVP
Hemophilia B	Factor IX
vWD	DDAVP, vWF+FVIII concentrate
ITP	Steroids, IVIG, Rituximab
DIC	FFP, cryoprecipitate, platelets
Liver disease	Vitamin K, FFP
Uremic bleeding	DDAVP, dialysis
TTP	Plasma exchange, steroids
Platelet dysfunction	Antifibrinolytics, platelet transfusion

🏥 Surgical Management of Bleeding Disorders

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📘 Overview

Surgical interventions in patients with bleeding disorders are generally:

• Not primary treatments for most bleeding disorders
• But may be necessary for managing complications, correcting anatomical defects, or in emergencies
• Always require pre-operative correction of bleeding tendencies

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🛑 Important Pre-Surgical Considerations

✅ Pre-surgical planning is critical to prevent excessive bleeding.

🧪 What to Check	✅ Action
Platelet count	Transfuse platelets if <50,000/μL before major surgery
Clotting profile (PT, aPTT, INR)	Correct with FFP, Vit K, specific factors
Specific factor deficiencies	Replace with factor concentrates (e.g., VIII, IX)
Type of bleeding disorder	Plan management accordingly
Consent and counseling	Must include bleeding risk

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🔹 1. Splenectomy

▶️ Indicated in:

• Chronic or refractory Immune Thrombocytopenic Purpura (ITP)
• Hypersplenism causing thrombocytopenia
• Hereditary spherocytosis (co-existing anemia + bleeding)

Surgical Notes:

• Laparoscopic preferred if possible
• Pre-op vaccinations: Pneumococcal, Meningococcal, H. influenzae
• Prophylactic antibiotics post-op
• Risk of post-splenectomy sepsis (OPSI)

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🔹 2. Surgical Control of Bleeding Sites

▶️ Used in:

• GI bleeding (e.g., variceal ligation, polypectomy, hemorrhoidectomy)
• Epistaxis (nasal packing, cautery, or vessel ligation)
• Recurrent bleeding lesions (e.g., bleeding fibroids → hysterectomy)

Management:

• Desmopressin (DDAVP) or factor support prior to surgery (esp. in Hemophilia, vWD)
• Maintain hemostasis intra-op with topical hemostats, cautery
• Local antifibrinolytics may be applied (e.g., tranexamic acid mouthwash for oral bleeding)

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🔹 3. Joint Surgery / Orthopedic Procedures (in Hemophilia)

▶️ Indicated for:

• Chronic hemarthrosis leading to joint deformity or disability
• Total joint replacement (hip/knee) in advanced cases

Management:

• Requires Factor VIII/IX coverage during perioperative period
• Use physiotherapy post-op to restore joint mobility
• Avoid IM injections and NSAIDs

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🔹 4. Emergency Surgeries in Bleeding Disorders

▶️ Situations may include:

• Trauma
• Ruptured ectopic pregnancy
• GI perforation or hemorrhage
• Intracranial bleed with raised ICP

Emergency Care Includes:

• Immediate blood and component transfusion
  ➤ PRBCs, FFP, cryoprecipitate, platelets as per need
• Maintain IV access, oxygenation, vitals
• Correct coagulopathy before/during surgery

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🔹 5. Liver Transplantation

▶️ For end-stage liver disease with unmanageable coagulopathy

• Restores liver’s synthetic function for clotting factors
• Requires comprehensive perioperative monitoring and critical care

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🧾 Other Surgical or Procedural Interventions

Procedure	Notes
Dental extractions	Needs pre-op factor support or DDAVP
Circumcision (in children with bleeding disorders)	Should only be done in centers with access to factor replacement
Central line placement	Ultrasound-guided, platelet count >50,000 needed
Endoscopy with biopsy	Requires factor coverage or platelet support if thrombocytopenic

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📌 Surgical Precautions in Bleeding Disorders

⚠️ Key Point	✅ Recommendation
Bleeding risk	Always correct platelets, factors before invasive procedures
Anesthesia	Regional anesthesia usually avoided in bleeding disorders
IM injections	Avoided — risk of hematoma
Surgical consent	Must explain increased bleeding risk
Post-op monitoring	Watch for internal/external bleeding signs

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✅ Summary Table: Surgical Interventions by Disorder

Disorder	Surgical Option	Purpose
ITP	Splenectomy	Increase platelet count
Hemophilia A/B	Joint surgery	Relieve hemarthrosis complications
Liver failure	Liver transplant	Restore clotting factor synthesis
Nasal bleeding	Nasal cautery/ligation	Stop uncontrolled epistaxis
GI bleeding	Endoscopy, surgery	Control active bleeding
GYN bleeding	Hysterectomy (rare)	Refractory menorrhagia in vWD
Tumors	Surgical removal	If causing thrombocytopenia or EPO excess

👩‍⚕️ Nursing Management of Bleeding Disorders

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🧠 I. Nursing Objectives

1. Prevent or control bleeding
2. Monitor for signs of hemorrhage or complications
3. Promote safety and comfort
4. Maintain adequate nutrition and fluid balance
5. Educate patient and family about disease, prevention, and self-care
6. Support psychosocial well-being

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📋 II. General Nursing Interventions

1️⃣ Assessment

• Vital signs: Monitor BP, HR, RR, SpO₂
• Inspect for bleeding signs:
  • Petechiae, ecchymosis, gum bleeding
  • Epistaxis, hematuria, melena
• Check neurological status: Monitor for signs of intracranial bleeding
• Assess for joint pain/swelling in hemophilia
• Daily weight and abdominal girth: Ascites or internal bleeding
• Monitor lab values: CBC, PT, aPTT, INR, platelet count

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2️⃣ Bleeding Prevention and Control

• Handle patient gently to avoid trauma
• Use soft toothbrushes, electric razors, and soft bedding
• Avoid invasive procedures unless necessary (e.g., IM injections, enemas)
• Apply direct pressure to bleeding sites
• Use cold compresses for nosebleeds or joint bleeding
• Administer medications (e.g., DDAVP, factor concentrates) as prescribed

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3️⃣ Medication Administration

• Ensure correct timing and dosage of:
  • Clotting factors (VIII, IX)
  • Platelets, FFP, or cryoprecipitate
  • Steroids, immunosuppressants (e.g., in ITP)
  • Desmopressin (DDAVP), vitamin K, antifibrinolytics
• Monitor for adverse reactions (e.g., allergic reaction to factor transfusion)

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4️⃣ Safety Precautions

• Fall prevention: padded side rails, assist with ambulation
• Keep emergency equipment nearby: oxygen, suction, crash cart
• Use small-gauge needles, minimize venipuncture
• Pad frequently used body parts (elbows, knees)
• Alert staff to high-bleeding-risk status (wristband or chart label)

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5️⃣ Nutritional Support

• Encourage iron-rich foods in chronic blood loss cases
• Monitor for anemia: pallor, fatigue, low Hb
• Adequate fluid intake to maintain circulation
• Avoid hard/crunchy foods that can irritate gums

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6️⃣ Psychosocial Support

• Provide emotional reassurance and anxiety reduction
• Address fears related to bleeding, transfusions, or surgery
• Support children and caregivers in chronic conditions like hemophilia

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🧾 III. Nursing Interventions by Specific Disorders

🔢 Disorder	👩‍⚕️ Nursing Focus
Hemophilia A/B	Administer factor VIII/IX, rest affected joints, apply cold packs, prevent trauma
ITP	Observe for mucosal bleeding, limit needle sticks, corticosteroid therapy, monitor platelets
vWD	Administer DDAVP, avoid aspirin/NSAIDs, educate on heavy menstrual bleeding
DIC	Manage shock, replace blood products (FFP, cryo, platelets), monitor organ function
Liver Disease	Support clotting factor replacement, monitor LFTs, prevent GI bleeding
CKD/Uremia	Administer DDAVP, prepare for dialysis, monitor bleeding time
Post-op care	Monitor surgical site for bleeding, teach wound care, pain control without NSAIDs

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📚 IV. Patient & Family Education

Topic	Content
Disease explanation	Nature, causes, importance of lifelong care
Bleeding precautions	Avoid contact sports, sharp objects, self-monitoring
Medication compliance	Importance of regular factor infusions, DDAVP, or steroids
Signs of serious bleeding	Severe headache, joint pain/swelling, dark stool/urine
Emergency plan	What to do during uncontrolled bleeding or trauma
Vaccinations	Especially in splenectomy cases (e.g., Pneumococcal vaccine)

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✅ V. Evaluation Criteria

• No active bleeding
• Stable vital signs and lab values (Hb, platelets, PT/aPTT)
• Patient demonstrates understanding of precautions
• Family participates in care
• Patient reports improved comfort and emotional support

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📌 Key Points for Nurses

• Bleeding disorders can be life-threatening if not managed properly
• Gentle care, timely medication, and preventive education are essential
• Always monitor for hidden bleeding signs (e.g., hematuria, hematemesis)
• Collaborate with physicians, hematologists, physiotherapists, and counselors
• Teach patients to carry bleeding disorder ID cards or medical alert bracelets

⚠️ Complications of Bleeding Disorders

Bleeding disorders, if left untreated or poorly managed, can lead to life-threatening complications, especially during surgery, trauma, or even routine activities. Complications depend on the type and severity of the disorder.

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🧠 I. Major Complications (All Types)

🔹 1. Severe or Life-Threatening Bleeding

• Hemorrhage following minor injury or surgery
• Internal bleeding (GI tract, retroperitoneum, brain)

🔹 2. Intracranial Hemorrhage

• Especially in Hemophilia A/B or severe ITP
• Can lead to seizures, coma, death

🔹 3. Hemarthrosis (Joint Bleeding)

• Common in Hemophilia
• Leads to joint deformities, pain, and permanent disability

🔹 4. Anemia

• Chronic blood loss → fatigue, pallor, low oxygen-carrying capacity

🔹 5. Shock

• Due to massive acute blood loss
• Requires urgent fluid, blood, and factor replacement

🔹 6. Infections

• Repeated transfusions → risk of hepatitis B/C, HIV
• Post-splenectomy → increased infection risk (OPSI)

🔹 7. Allergic or Anaphylactic Reactions

• From blood component transfusions or factor infusions

🔹 8. Complications of Medications

• Steroid side effects: weight gain, hyperglycemia, infection
• Immunosuppressants: increased infection risk
• Heparin/Warfarin: risk of over-anticoagulation → bleeding

🔹 9. DIC (Disseminated Intravascular Coagulation)

• In patients with infections, trauma, sepsis
• Clotting → consumption of platelets/factors → bleeding and organ failure

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📌 Key Points to Remember: Bleeding Disorders

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🔸 General Understanding

✅ Bleeding disorders result from platelet problems, clotting factor deficiency, or vascular defects
✅ May be inherited (e.g., Hemophilia, vWD) or acquired (e.g., liver disease, DIC)

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🔸 Assessment & Monitoring

✅ Watch for petechiae, bruising, gum bleeds, joint swelling, blood in urine/stool
✅ Monitor CBC, platelet count, PT, aPTT, INR
✅ Evaluate for signs of internal bleeding (tachycardia, low BP, restlessness)

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🔸 Management Essentials

✅ Administer factor concentrates, platelets, FFP, vitamin K, or DDAVP as per type
✅ Always treat underlying cause in acquired disorders
✅ Use gentle care practices to avoid trauma

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🔸 Patient Safety

✅ Avoid aspirin, NSAIDs, IM injections, and trauma
✅ Provide soft oral care, padded rails, non-slip footwear
✅ Use small-gauge needles, apply pressure post-venipuncture

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🔸 Education & Self-Care

✅ Teach patient to report any unusual bleeding
✅ Encourage use of medical alert bracelet
✅ Provide vaccines (especially post-splenectomy)
✅ Explain long-term therapy compliance (e.g., factor infusions in Hemophilia)

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🔸 Multidisciplinary Approach

✅ Involve hematologist, nurse, physiotherapist, counselor
✅ Coordinate care during surgery, dental work, or childbirth

🧬 Clotting Factor Defects

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📘 Definition

Clotting factor defects are disorders of the coagulation system where there is either a deficiency or dysfunction of one or more clotting factors, leading to delayed or abnormal blood clot formation and an increased risk of prolonged bleeding.

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❓ Causes

🔹 A. Inherited Causes

• Genetic mutations affecting synthesis or function of specific factors

Disorder	Deficient Factor
Hemophilia A	Factor VIII
Hemophilia B (Christmas disease)	Factor IX
Hemophilia C	Factor XI
Von Willebrand Disease	vWF (also stabilizes Factor VIII)
Rare inherited deficiencies	Factor I, II, V, VII, X, XIII (very rare)

🔹 B. Acquired Causes

• Result from diseases or medications affecting factor production or activity

Cause	Mechanism
Vitamin K deficiency	Needed for Factors II, VII, IX, X
Liver disease	Liver synthesizes most factors
Warfarin therapy	Inhibits vitamin K-dependent factors
DIC (Disseminated Intravascular Coagulation)	Consumption of clotting factors
Massive transfusions	Dilutional coagulopathy

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🔠 Types of Clotting Factor Defects

Type	Description
Hemophilia A (classic)	X-linked; ↓ Factor VIII
Hemophilia B (Christmas)	X-linked; ↓ Factor IX
Hemophilia C	Autosomal recessive; ↓ Factor XI
Von Willebrand Disease	Autosomal dominant/recessive; ↓ vWF and Factor VIII
Rare Deficiencies	Fibrinogen (I), Prothrombin (II), Factor V, VII, X, XIII, etc.
Acquired Coagulopathy	From liver disease, warfarin, DIC, etc.

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🧬 Pathophysiology

1. Injury → Vessel damage → triggers coagulation cascade
2. Clotting factors activate sequentially to convert fibrinogen → fibrin
3. If any clotting factor is deficient/dysfunctional → interruption in clotting cascade
4. Results in:
   • Prolonged bleeding
   • Inability to stabilize platelet plug
   • Hemarthrosis, hematoma, mucosal bleeding

Primary hemostasis may be intact (platelet plug), but secondary hemostasis (fibrin clot formation) is impaired.

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⚠️ Signs and Symptoms

🩸 General Signs	🧠 Clinical Details
Prolonged bleeding	After cuts, surgery, dental procedures
Hemarthrosis	Bleeding into joints – pain, swelling, deformity (common in Hemophilia A/B)
Hematomas	Deep muscle or soft tissue bleeding
Mucosal bleeding	Nosebleeds, gum bleeding (esp. in vWD)
Hematuria, melena	Blood in urine or stools
Postpartum hemorrhage	Seen in undiagnosed mild disorders

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🔬 Diagnosis

Test	Purpose
CBC	May show anemia from chronic blood loss
PT (Prothrombin Time)	↑ in Factor VII, II, V, X, fibrinogen deficiency
aPTT (Activated Partial Thromboplastin Time)	↑ in Hemophilia A/B, vWD
Bleeding Time	↑ in vWD and platelet dysfunction
Mixing Studies	Differentiate between factor deficiency and inhibitor
Factor Assay Tests	Confirm type and severity (e.g., ↓ Factor VIII in Hemophilia A)
Von Willebrand panel	vWF antigen and activity levels
Liver function tests	For acquired clotting defects
Genetic testing	In hereditary cases

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💊 Medical Management

Disorder	Treatment
Hemophilia A	Recombinant/purified Factor VIII, Desmopressin (mild cases)
Hemophilia B	Factor IX concentrate
Hemophilia C	FFP (no specific concentrate available)
vWD	Desmopressin (Type 1), vWF+Factor VIII concentrate (Types 2, 3)
Vitamin K Deficiency	Vitamin K IM/IV, dietary correction
Liver Disease	FFP, vitamin K, treat liver cause
DIC	Treat underlying cause, replace FFP, platelets, cryoprecipitate

Supportive Treatments:

• Antifibrinolytics (e.g., tranexamic acid)
• Avoid aspirin/NSAIDs
• Immunosuppressants in acquired hemophilia

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🏥 Surgical Management

• Surgical treatment is not curative, but may be required for:
  • Joint deformities from hemarthrosis → joint replacement
  • Uncontrolled bleeding → cauterization, splenectomy (in selected cases)
• Pre-surgical factor coverage mandatory
• Desmopressin for minor procedures in mild Hemophilia A and vWD
• Avoid regional anesthesia unless factor levels are normalized

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👩‍⚕️ Nursing Management

🔹 Task	🩺 Nursing Actions
Assessment	Monitor for bleeding signs (joints, gums, skin, stool, urine)
Safe handling	Use soft toothbrush, avoid trauma, padded side rails
Medication care	Administer factor infusions, monitor for reactions
Bleeding control	Apply pressure, cold compress, elevate limb
Patient education	Avoid NSAIDs, teach self-infusion (home therapy), ID card usage
Nutrition	Iron-rich diet if anemic, avoid alcohol in liver-related disorders

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⚠️ Complications

🚨 Complication	Explanation
Hemarthrosis	Joint bleeding → chronic pain and disability
Anemia	Chronic blood loss
Intracranial hemorrhage	Potentially fatal, especially in trauma
Inhibitor development	Antibodies to factor therapy (in hemophilia)
Transfusion reactions	Allergy, infection risk
Psychosocial issues	Anxiety, fear of injury, poor quality of life

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📌 Key Points to Remember

✅ Clotting factor defects → impaired secondary hemostasis
✅ Hemophilia A = Factor VIII; Hemophilia B = Factor IX; vWD = vWF + Factor VIII
✅ Management: Factor replacement, DDAVP (in mild cases), antifibrinolytics
✅ Avoid IM injections, NSAIDs, and trauma
✅ Lifelong management needed in inherited cases
✅ Educate patient on self-care, infusion techniques, and emergency recognition
✅ Regular follow-up with hematologist is essential

🧬 Platelet Defects

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📘 Definition

Platelet defects refer to abnormalities in the number or function of platelets, resulting in impaired primary hemostasis (the initial step in blood clot formation). These defects cause a tendency toward prolonged bleeding, especially from mucous membranes and skin, even with minor trauma.

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❓ Causes of Platelet Defects

Platelet defects are broadly classified into:

🔹 A. Quantitative Defects (Thrombocytopenia)

Low platelet count (<150,000/μL)

Cause	Description
Decreased production	Bone marrow failure (e.g., aplastic anemia, leukemia, chemotherapy, radiation)
Increased destruction	Immune causes (ITP), DIC, infections (e.g., dengue, HIV)
Sequestration	Hypersplenism – spleen traps more platelets
Drug-induced	Heparin-induced thrombocytopenia (HIT), sulfa drugs
Nutritional deficiency	Vitamin B12, folate

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🔹 B. Qualitative Defects (Platelet Dysfunction)

Normal platelet count but defective function

Cause	Description
Inherited disorders	Glanzmann’s thrombasthenia (defective aggregation), Bernard-Soulier syndrome (defective adhesion)
Acquired dysfunction	Uremia (CKD), liver disease, myeloproliferative disorders
Drug-induced	Aspirin, NSAIDs, clopidogrel (inhibit platelet function)

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🔠 Types of Platelet Defects

Type	Description	Platelet Count
Immune Thrombocytopenic Purpura (ITP)	Autoimmune destruction of platelets	Low
Thrombotic Thrombocytopenic Purpura (TTP)	Microthrombi use up platelets	Low
Heparin-Induced Thrombocytopenia (HIT)	Immune reaction to heparin	Low
Drug-induced thrombocytopenia	Drugs destroy platelets or suppress production	Low
Inherited qualitative defects	Platelet count normal but dysfunction (e.g., Glanzmann’s)	Normal
Acquired dysfunction (uremia, liver disease)	Platelet count normal; function impaired	Normal

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🧠 Summary

Category	Examples
Low Platelet Count (Thrombocytopenia)	ITP, TTP, HIT, leukemia
Normal Count but Dysfunction	Glanzmann’s, uremia, aspirin use

I. 🧬 Pathophysiology of Platelet Defects

Platelets are essential for primary hemostasis, forming the initial plug at a site of vascular injury. Defects in their number or function lead to:

• Impaired clot formation
• Prolonged bleeding
• Increased bruising and mucosal bleeding

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🔹 A. Quantitative Defects – Thrombocytopenia (↓ Platelet Count)

Mechanism	Explanation
Decreased production	Bone marrow disorders (e.g., leukemia, aplastic anemia) → ↓ platelet formation
Increased destruction	Immune (ITP), drugs, infections → body destroys platelets prematurely
Sequestration	Enlarged spleen (hypersplenism) stores excessive platelets
Consumption	DIC, TTP → platelets used up in widespread clotting

➡️ Result: Inadequate number of platelets → failure of primary hemostatic plug formation → prolonged bleeding time

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🔹 B. Qualitative Defects – Platelet Dysfunction (Normal Count)

Mechanism	Explanation
Inherited dysfunction	Structural or receptor defects on platelets (e.g., Glanzmann’s thrombasthenia – defective aggregation)
Acquired dysfunction	Uremia, liver failure, drug-induced (aspirin, NSAIDs) interfere with platelet adhesion/aggregation

➡️ Result: Platelets are present but cannot adhere or aggregate → bleeding despite normal count

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II. ⚠️ Signs and Symptoms of Platelet Defects

🩸 General Symptoms (Common to All Types)

System	Symptoms
Skin	Easy bruising, petechiae (pinpoint red spots), purpura
Mucosa	Bleeding gums, epistaxis (nosebleeds), heavy menstruation
GI/GU	Hematuria (blood in urine), melena (black stools)
Post-Injury/Surgery	Prolonged bleeding, slow clotting
Neurological (in severe cases)	Headache, seizures (if intracranial bleeding)

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📌 Specific Clues by Type

Type	Key Clinical Features
ITP	Sudden onset petechiae/purpura in child or adult, low platelet count, no other systemic illness
TTP	Fever, renal failure, neurological signs, thrombocytopenia
Drug-induced	Bleeding after starting new medication (e.g., heparin, antibiotics)
Uremic platelet dysfunction	Bleeding in CKD patients with normal platelet count
Glanzmann’s thrombasthenia	Lifelong mucosal bleeding, poor platelet aggregation on lab testing

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III. 🔬 Diagnosis of Platelet Defects

🧪 Basic Blood Tests

Test	Purpose
CBC (Complete Blood Count)	Detects ↓ platelet count (thrombocytopenia)
Peripheral Blood Smear	Assesses platelet size, morphology, any abnormal cells
Bleeding Time (BT)	Prolonged in both qualitative and quantitative defects
Clotting Time (CT)	May be normal unless DIC present
PT/aPTT	Usually normal in isolated platelet defects (prolonged in DIC, liver disease)

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🧪 Specific Tests

Test	Used For
Platelet Aggregation Studies	Identifies functional defects (e.g., Glanzmann’s, drug-induced)
Flow Cytometry	Detects receptor abnormalities on platelets
Bone Marrow Aspiration	If platelet production issue is suspected (e.g., leukemia, aplasia)
Platelet Antibody Test	For immune thrombocytopenia (ITP)
Urea/Creatinine	To detect uremic bleeding in CKD
Liver Function Test (LFT)	Rule out liver disease-related platelet dysfunction
D-dimer, FDPs, Fibrinogen	To assess for DIC in consumptive coagulopathies
Heparin-PF4 Antibody (HIT test)	To diagnose Heparin-Induced Thrombocytopenia

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🧠 Diagnostic Clues by Condition

Condition	Key Lab Findings
ITP	↓ platelets, normal PT/aPTT, ↑ bleeding time
TTP	↓ platelets, schistocytes on smear, renal failure
HIT	↓ platelets after heparin, positive PF4 antibody
Glanzmann’s	Normal count, defective aggregation
Uremia	Normal count, prolonged bleeding time, CKD on labs

💊🩸 Medical and Surgical Management of Platelet Defects

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🧠 I. Medical Management

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🔹 A. Quantitative Platelet Defects (Thrombocytopenia)

Platelet count <150,000/μL

1️⃣ Immune Thrombocytopenic Purpura (ITP)

Treatment	Purpose
Corticosteroids (e.g., Prednisolone)	First-line to reduce immune-mediated platelet destruction
IV Immunoglobulin (IVIG)	Rapid platelet increase; used in emergencies or before surgery
Anti-D Immunoglobulin	For Rh+ patients who are not splenectomized
Rituximab (Monoclonal antibody)	For steroid-resistant or chronic cases
Platelet transfusion	Reserved for severe bleeding or surgery
TPO agonists (e.g., Eltrombopag)	Stimulate platelet production in refractory cases

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2️⃣ Thrombotic Thrombocytopenic Purpura (TTP)

Treatment	Notes
Plasma Exchange (Plasmapheresis)	First-line therapy; removes autoantibodies
Corticosteroids	Reduce immune activity
Rituximab	For refractory TTP
Caplacizumab	New drug; inhibits vWF-platelet interaction (if available)
Avoid platelet transfusion	May worsen microthrombi formation unless life-threatening bleeding exists

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3️⃣ Drug-Induced Thrombocytopenia (e.g., Heparin-Induced Thrombocytopenia – HIT)

Treatment	Notes
Discontinue heparin	Immediate action required
Start alternative anticoagulants	E.g., Argatroban or Fondaparinux
Avoid platelet transfusion	Unless active bleeding

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4️⃣ Thrombocytopenia in Bone Marrow Suppression (e.g., Leukemia, Aplastic Anemia)

Treatment	Notes
Platelet transfusion	For active bleeding or prophylactically when <10,000/μL
Treat underlying condition	Chemotherapy, immunosuppression, or transplant

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🔹 B. Qualitative Platelet Defects (Normal count but dysfunctional)

1️⃣ Inherited Disorders (e.g., Glanzmann’s Thrombasthenia, Bernard-Soulier Syndrome)

Treatment	Notes
Platelet transfusion	For bleeding or surgery
Antifibrinolytics (Tranexamic acid, EACA)	For mucosal bleeding, dental procedures
Recombinant Factor VIIa (rFVIIa)	Used in Glanzmann’s during severe bleeding or surgery
Avoid aspirin/NSAIDs	These worsen platelet function

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2️⃣ Acquired Platelet Dysfunction (Uremia, Liver Disease, Drug-Induced)

Cause	Treatment
Uremia (CKD)	Desmopressin (DDAVP), dialysis, antifibrinolytics
Liver disease	Treat liver cause, vitamin K, FFP or platelet transfusion
Drug-induced (aspirin, clopidogrel)	Discontinue offending drug, use platelet transfusion if needed

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🏥 II. Surgical Management

Platelet defects are not usually treated surgically, but surgery may be required to manage complications or to treat bleeding sources.

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🔹 A. Splenectomy

✅ Indicated for:

• Chronic or refractory Immune Thrombocytopenic Purpura (ITP)
• Hypersplenism causing platelet sequestration

📌 Pre/Post-op Care:

• Vaccinate against pneumococcus, meningococcus, and H. influenzae (2 weeks before surgery)
• Lifelong risk of infection → prophylactic antibiotics
• Post-op monitoring for bleeding/infection

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🔹 B. Emergency Surgical Interventions for Active Bleeding

Site	Intervention
Epistaxis (Nosebleeds)	Nasal packing, cautery, arterial ligation if severe
GI Bleeding	Endoscopy with cautery, polypectomy, or surgery
Menorrhagia (heavy periods)	Endometrial ablation, hysterectomy in severe chronic cases
Intracranial Hemorrhage	Neurosurgical evacuation if life-threatening

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🔹 C. Other Procedures

Procedure	Consideration
Dental Extraction, Minor Surgery	Requires pre-procedure platelet transfusion or antifibrinolytics
Joint Surgery (if hemarthrosis occurs)	Performed rarely; ensure platelet and factor coverage
Bone Marrow Transplant	For platelet production failure (e.g., aplastic anemia, leukemia)

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📌 Summary Table: Management at a Glance

Type of Defect	Medical Treatment	Surgical Role
ITP	Steroids, IVIG, TPO agonists	Splenectomy (chronic cases)
TTP	Plasma exchange, steroids	Avoid surgery unless life-threatening
HIT	Stop heparin, switch anticoagulant	No role for surgery
Inherited dysfunction	Platelets, antifibrinolytics, rFVIIa	None, except bleeding source control
Uremia-related	DDAVP, dialysis	None specific
GI/Nasal bleeding	Antifibrinolytics ± transfusion	Cauterization or ligation
Menorrhagia	Hormonal or antifibrinolytics	Hysterectomy (last resort)

👩‍⚕️ Nursing Management of Platelet Defects

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🧠 I. Nursing Objectives

1. Prevent and manage bleeding episodes
2. Monitor for complications like anemia or hemorrhage
3. Administer medications and blood products safely
4. Educate patient and family about precautions and self-care
5. Promote comfort, emotional well-being, and injury prevention

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📋 II. General Nursing Interventions

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1️⃣ Assessment

What to Assess	Why
Vital signs	Monitor for hypotension, tachycardia → signs of bleeding
Skin & mucous membranes	Look for petechiae, ecchymosis, gum/nasal bleeding
Stool and urine	Check for melena or hematuria
Neurological status	Assess for headache, confusion → possible brain bleed
Lab reports	Platelet count, bleeding time, PT/aPTT, CBC, renal/liver function

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2️⃣ Bleeding Prevention

Action	Purpose
Use soft toothbrush & electric razor	Prevent gum and skin injury
Avoid IM injections and rectal procedures	Prevent internal hematoma
Apply pressure after venipuncture	Promote clot formation
Use small-gauge needles	Reduce trauma to veins
Encourage gentle activities	Prevent bruises or trauma
Padded bed rails, fall precautions	Prevent injury in low platelet count

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3️⃣ Medication & Transfusion Care

Medication	Nursing Role
Steroids (e.g., Prednisolone)	Monitor glucose, infection signs
IVIG	Monitor for fever, allergic reaction
Eltrombopag or Rituximab	Observe for adverse reactions
Platelet transfusion	Administer as prescribed, observe for reactions
Antifibrinolytics (e.g., Tranexamic acid)	Give for mucosal bleeds or surgery
Discontinue aspirin/NSAIDs	Educate patient not to take without consulting physician

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4️⃣ Nutrition and Hydration

• Provide iron-rich foods (e.g., spinach, meat, dates) if anemia is present
• Encourage adequate hydration
• Avoid alcohol (can worsen platelet production and function)

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5️⃣ Infection Control (especially post-splenectomy or on immunosuppressants)

• Monitor temperature daily
• Practice hand hygiene
• Administer vaccines (if splenectomy)
• Educate about signs of infection and when to seek help

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📚 III. Patient & Family Education

Topic	Key Teaching Points
Disease explanation	Nature of platelet defect, need for regular monitoring
Bleeding precautions	Avoid trauma, use soft tools, report any unusual bleeding
Medication compliance	Importance of steroids, IVIG, TPO agents, or other meds
Emergency signs	Headache, vomiting, sudden weakness → possible internal bleeding
Medication safety	Avoid aspirin, NSAIDs, herbal supplements without doctor’s advice
Medical alert	Wear ID band or carry emergency information card

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✅ IV. Evaluation Criteria

• No signs of active bleeding
• Vital signs remain stable
• Patient understands and applies safety precautions
• Platelet count improves or is maintained
• Patient/family can correctly describe when to seek help
• Medication side effects are managed or prevented

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📌 Quick Nursing Tips

✅ Always treat even minor bleeding seriously in thrombocytopenic patients
✅ Do not give IM injections or rectal meds
✅ Educate patient to avoid contact sports or risky activities
✅ Coordinate with the medical team before any planned surgery or dental work
✅ Emotional support is important—help patients cope with lifestyle changes

⚠️ Complications of Platelet Defects

Platelet defects (both quantitative and qualitative) can lead to serious, even life-threatening complications if not identified and managed properly.

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🧠 I. Major Complications

🚨 Complication	📋 Description
Spontaneous bleeding	Especially when platelet count <20,000/μL – may occur without trauma
Mucosal hemorrhage	Persistent gum bleeding, epistaxis, heavy menstruation
GI bleeding	Can lead to melena, hematemesis, anemia
Hematuria	Blood in urine; may cause pain, clot retention
Intracranial hemorrhage	Life-threatening; may present with headache, vomiting, altered consciousness
Hemarthrosis (in dysfunction disorders)	Repeated joint bleeding → pain, swelling, deformity (less common than in hemophilia)
Anemia	Due to chronic or acute blood loss
Infection risk (esp. post-splenectomy or on steroids)	Increased susceptibility to bacterial infections
Allergic transfusion reactions	To platelet or blood product infusions
Emotional & psychosocial impact	Fear of bleeding, activity restriction, depression/anxiety in chronic cases

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📌 II. Key Points for Nursing & Clinical Practice

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🔸 Understanding the Disorder

✅ Platelet defects can result from low count (thrombocytopenia) or poor function
✅ They affect primary hemostasis, leading to prolonged bleeding time

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🔸 Assessment and Monitoring

✅ Watch for petechiae, purpura, mucosal bleeds, hematuria, melena
✅ Monitor platelet count, bleeding time, and vital signs
✅ Be vigilant for neurological signs → possible brain bleed

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🔸 Prevention and Precautions

✅ Avoid IM injections, rough handling, invasive procedures unless necessary
✅ Use soft toothbrushes, electric razors, and pad sharp surfaces
✅ Apply pressure after venipuncture or injections

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🔸 Medication and Transfusions

✅ Administer platelet transfusions as ordered (usually <10,000/µL or active bleeding)
✅ Use steroids, IVIG, antifibrinolytics, or TPO agents per physician order
✅ Avoid NSAIDs, aspirin, herbal meds that impair platelet function

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🔸 Patient Education

✅ Teach signs of serious bleeding and when to seek help
✅ Encourage compliance with treatment and follow-up
✅ Advise wearing medical alert bracelets
✅ Post-splenectomy → vaccination + infection prevention measures

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🔸 Holistic Care

✅ Support emotional well-being, especially in children/adolescents with chronic platelet issues
✅ Include family in education and care planning
✅ Coordinate with a multidisciplinary team for best outcomes

🧬 Thalassemia

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📘 Definition

Thalassemia is an inherited blood disorder characterized by abnormal or reduced synthesis of hemoglobin chains (alpha or beta), leading to chronic hemolytic anemia.

• Hemoglobin (Hb) is made up of:
  • 4 globin chains (2 alpha + 2 beta in adults = HbA)
  • Heme group (iron-containing portion)

In thalassemia, the body fails to produce adequate amounts of one or more globin chains, which results in:

• Ineffective erythropoiesis
• RBC destruction (hemolysis)
• Chronic anemia

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❓ Causes of Thalassemia

✅ Primary Cause: Genetic Mutation

• Thalassemia is inherited in an autosomal recessive pattern
• Caused by mutations or deletions in the genes responsible for alpha or beta globin chains

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🔹 Types Based on Chain Affected

Type	Gene Affected	Region Common
Alpha Thalassemia	Deletion in alpha-globin genes on chromosome 16	Southeast Asia, Africa, Middle East
Beta Thalassemia	Mutation in beta-globin gene on chromosome 11	India, Pakistan, Mediterranean, Middle East

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🔹 Inheritance Patterns

Condition	Genes Affected	Severity
Thalassemia Trait (Minor)	1 defective gene	Mild or no symptoms
Thalassemia Intermedia	2 defective genes (less severe mutation)	Moderate symptoms
Thalassemia Major (Cooley’s Anemia)	2 severe defective genes	Severe, life-threatening anemia requiring regular transfusions

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🔹 Risk Factors

• Family history of thalassemia
• Ethnicity: common in South Asian, Mediterranean, Middle Eastern, African populations
• Consanguineous marriage (increased risk of inheriting two defective genes)

🧬 Types of Thalassemia.

Thalassemias are genetic disorders characterized by reduced or absent synthesis of alpha or beta globin chains of hemoglobin.

They are broadly classified into:

✅ 1. Alpha Thalassemia

✅ 2. Beta Thalassemia

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1️⃣ Alpha Thalassemia

📘 Cause:

• Mutation or deletion of alpha-globin genes on chromosome 16
• Normally, there are 4 alpha-globin genes (2 from each parent)

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🩸 Types of Alpha Thalassemia (Based on number of gene deletions)

Type	Genes Deleted	Clinical Severity	Features
Silent Carrier	1 gene	No symptoms	Normal Hb, no anemia
Alpha Thalassemia Trait (Minor)	2 genes	Mild microcytic anemia	Often asymptomatic, detected incidentally
Hemoglobin H Disease (HbH)	3 genes	Moderate to severe	Chronic hemolytic anemia, hepatosplenomegaly
Hydrops Fetalis (Hb Bart’s)	4 genes	Lethal in utero	Severe anemia, heart failure, death before or shortly after birth

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2️⃣ Beta Thalassemia

📘 Cause:

• Mutation in the beta-globin gene on chromosome 11
• Normally, there are 2 beta-globin genes (1 from each parent)

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🩸 Types of Beta Thalassemia

Type	Gene Mutation	Clinical Severity	Features
Beta Thalassemia Trait (Minor)	1 defective gene	Mild	Mild microcytic anemia, often asymptomatic
Beta Thalassemia Intermedia	2 partially defective genes	Moderate	Anemia, occasional transfusions, bone deformities
Beta Thalassemia Major (Cooley’s Anemia)	2 severely mutated genes	Severe	Severe anemia from infancy, growth retardation, requires lifelong transfusions

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✅ Comparison of Alpha vs Beta Thalassemia

Feature	Alpha Thalassemia	Beta Thalassemia
Gene Affected	Alpha-globin (chromosome 16)	Beta-globin (chromosome 11)
Types	Silent carrier, Trait, HbH, Hydrops fetalis	Trait, Intermedia, Major
Inheritance	Autosomal recessive	Autosomal recessive
Common in	Southeast Asia, Africa	Mediterranean, South Asia
Severe Form	HbH disease, Hydrops fetalis	Beta Thalassemia Major

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🧠 Note on Hemoglobin Variants in Thalassemia

• HbH: Formed in alpha thalassemia (excess beta chains)
• Hb Bart’s: Seen in hydrops fetalis (gamma tetramers)
• HbF (Fetal Hemoglobin): Elevated in beta thalassemia major
• HbA2: Often elevated in beta thalassemia trait

🧬 Pathophysiology of Thalassemia.

Thalassemia is a hereditary disorder of hemoglobin synthesis caused by reduced or absent production of one or more globin chains (alpha or beta). This leads to:

• Imbalanced globin chain production
• Defective hemoglobin
• Hemolysis (destruction of RBCs)
• Chronic anemia

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1️⃣ Alpha Thalassemia – Pathophysiology

🔬 Genetic Basis:

• Mutation or deletion in alpha-globin genes on chromosome 16
• Normally 4 alpha-globin genes (2 from each parent)

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🧬 Pathological Process:

Condition	Pathophysiological Sequence
Silent Carrier (1 gene deleted)	Normal Hb synthesis → no clinical effect
Alpha Thalassemia Trait (2 genes deleted)	↓ Alpha chains → mild imbalance → mild microcytic anemia
Hemoglobin H Disease (3 genes deleted)	Marked ↓ alpha chains → excess β chains → form unstable HbH (β₄) → RBC membrane damage → hemolysis → moderate to severe anemia
Hydrops Fetalis (4 genes deleted)	No alpha chain synthesis → excess γ chains form Hb Bart’s (γ₄) → extremely high oxygen affinity → severe hypoxia → fetal hydrops, heart failure, and death

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2️⃣ Beta Thalassemia – Pathophysiology

🔬 Genetic Basis:

• Mutation in beta-globin gene on chromosome 11
• Normally 2 beta-globin genes (1 from each parent)

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🧬 Pathological Process:

Condition	Pathophysiological Sequence
Beta Thalassemia Trait (1 gene defective)	↓ β-chain synthesis → mild imbalance → ↑ HbA₂ or HbF → mild anemia
Beta Thalassemia Intermedia (2 mild mutations)	↓↓ β-chains → ↑ unpaired alpha chains → alpha chain precipitation → RBC membrane damage → ineffective erythropoiesis and hemolysis → moderate anemia
Beta Thalassemia Major (Cooley’s Anemia)	Almost no β-chain synthesis → ↑ unpaired alpha chains → severe membrane damage, premature RBC death in marrow → ineffective erythropoiesis, severe hemolysis → severe anemia, extramedullary hematopoiesis, skeletal deformities, iron overload from transfusions

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🔁 Common Features in All Types

🔬 Process	Result
Imbalanced globin chain synthesis	Unstable Hb → hemolysis
Destruction of RBC precursors in bone marrow (ineffective erythropoiesis)	Anemia despite increased erythropoiesis
Increased iron absorption and transfusions	Risk of iron overload (hemochromatosis) → liver, heart, endocrine damage
Bone marrow expansion	Due to increased erythropoietin → skeletal deformities, facial changes
Splenomegaly	Due to hemolysis and extramedullary hematopoiesis

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🧠 Summary of Pathophysiology (Flowchart Format)

1. 🧬 Genetic mutation → ↓ or absent alpha/beta globin chain
2. ⚖️ Imbalance of globin chains → unstable hemoglobin
3. 🔥 Ineffective erythropoiesis in bone marrow → RBCs destroyed before entering circulation
4. 🩸 Hemolysis of circulating RBCs → chronic anemia
5. ⬆️ Increased erythropoietin → marrow hyperplasia
6. 🏥 Blood transfusions + ⬆️ iron absorption → iron overload
7. ❗ Complications: growth retardation, skeletal changes, organ damage

⚠️ Signs and Symptoms of Thalassemia

Symptoms vary based on the type (Minor, Intermedia, Major) and severity of the disease.

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✅ 1. Thalassemia Minor (Trait / Carrier)

🩺 Symptoms	Notes
Often asymptomatic	Discovered incidentally
Mild fatigue	In cases with mild anemia
Slight pallor	May resemble iron-deficiency anemia
No organomegaly	Spleen/liver usually normal

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⚠️ 2. Thalassemia Intermedia

🩺 Symptoms	Notes
Moderate anemia	Fatigue, weakness, exercise intolerance
Pallor, jaundice	Due to chronic hemolysis
Splenomegaly	Enlarged spleen due to RBC destruction
Bone pain or facial changes	Mild marrow expansion in long-term cases
Growth delay (in children)	Due to chronic anemia

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🚨 3. Thalassemia Major (Cooley’s Anemia)

Appears in infancy (3–6 months) as fetal Hb declines

🩺 Symptoms	Notes
Severe anemia	Pallor, irritability, poor feeding
Jaundice	Due to ongoing hemolysis
Failure to thrive	Poor growth, developmental delay
Hepatosplenomegaly	Massive due to hemolysis and extramedullary hematopoiesis
Bone deformities	Frontal bossing, “chipmunk facies”, due to marrow expansion
Dark urine	Due to hemolysis byproducts
Frequent infections	Due to splenomegaly and immune suppression
Signs of iron overload	Due to repeated transfusions (later stages): bronze skin, diabetes, heart failure

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🔬 Diagnosis of Thalassemia

Diagnosis includes clinical history, physical examination, and lab investigations.

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🧪 1. Blood Tests

Test	Findings
CBC (Complete Blood Count)	↓ Hb, ↓ MCV, ↓ MCH (microcytic hypochromic anemia)
Peripheral blood smear	Target cells, anisopoikilocytosis, nucleated RBCs
Reticulocyte count	Normal or ↑ (due to ongoing hemolysis)
Serum iron, ferritin, TIBC	Usually normal or ↑ (helps rule out iron-deficiency anemia)

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🧬 2. Hemoglobin Electrophoresis (Confirmatory Test)

Thalassemia Type	Electrophoresis Result
Beta Thalassemia Trait	↑ HbA₂ (>3.5%), slight ↑ HbF
Beta Thalassemia Major	↑↑ HbF (>90%), ↓/absent HbA, ↑ HbA₂
Alpha Thalassemia Trait	Often normal, may show minor ↓ HbA
HbH disease (alpha)	Presence of HbH (β₄) band
Hydrops fetalis (Hb Bart’s)	Detects Hb Bart’s (γ₄)

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🧪 3. Other Investigations

Test	Purpose
Genetic testing	Confirms mutation in alpha or beta genes
LFT, RFT	Assess liver/kidney damage (especially in iron overload)
Serum ferritin	Measures iron stores (↑ in iron overload)
MRI T2 (Liver/Heart)	Detects iron deposition in tissues
Ultrasound	Detects hepatosplenomegaly
Bone X-ray/Skull	“Hair-on-end” appearance in chronic marrow expansion (beta thalassemia major)

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👩‍⚕️ Special Diagnostic Considerations

• Family history: Commonly positive in inherited thalassemia
• Neonatal screening: May help detect major cases early
• Prenatal diagnosis: Chorionic villus sampling or amniocentesis for high-risk couples

💊 Medical Management of Thalassemia.

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1️⃣ Thalassemia Minor (Trait)

Usually asymptomatic or presents with mild anemia

✅ Management:

• No specific treatment required
• Regular monitoring of hemoglobin and iron status
• Folic acid supplementation (to support RBC production)
• Genetic counseling for family planning
• Avoid unnecessary iron supplements unless iron deficiency is proven
• Encourage nutritional counseling with iron-rich foods (only if needed)

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2️⃣ Thalassemia Intermedia

Moderate anemia, does not always require regular transfusions

✅ Management Goals:

• Maintain adequate Hb
• Prevent complications (iron overload, bone deformities)

✅ Treatment Options:

• Occasional blood transfusions: During illness, surgery, pregnancy, or growth spurts
• Folic acid supplements: To enhance erythropoiesis
• Hydroxyurea: Stimulates production of fetal hemoglobin (HbF), which improves anemia
• Iron chelation therapy (if iron overload occurs):
  • Deferoxamine (IV/SC)
  • Deferasirox or Deferiprone (oral agents)
• Monitor for:
  • Splenomegaly
  • Growth retardation
  • Bone changes

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3️⃣ Thalassemia Major (Cooley’s Anemia)

Severe, transfusion-dependent anemia diagnosed in infancy

✅ Aims of Treatment:

• Maintain Hb >9–10 g/dL
• Prevent iron overload
• Support growth and development
• Prevent/reduce complications

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🔹 A. Regular Blood Transfusions

• Given every 2–4 weeks
• Use leukocyte-depleted, cross-matched packed RBCs
• Target: Keep Hb >9 g/dL to suppress bone marrow expansion
• Monitor for transfusion reactions and alloimmunization

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🔹 B. Iron Chelation Therapy (to prevent iron overload from transfusions)

Drug	Route	Notes
Deferoxamine	Subcutaneous/IV	Used with infusion pump, painful in children
Deferasirox	Oral	Once daily, convenient
Deferiprone	Oral	Used alone or with deferoxamine

▶️ Monitor serum ferritin every 3 months
▶️ MRI T2 to assess liver/heart iron overload

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🔹 C. Folic Acid Supplementation

• Supports erythropoiesis
• Usually given daily (1–5 mg)

---

🔹 D. Hydroxyurea

• Increases fetal hemoglobin (HbF)
• Reduces transfusion requirement in some patients

---

🔹 E. Endocrine and Nutritional Support

• Monitor for diabetes, hypothyroidism, delayed puberty
• Nutritional therapy for growth and immunity
• Vitamin D, calcium supplementation to prevent osteoporosis

---

🔹 F. Vaccination and Infection Prevention

• Hepatitis B, Hepatitis C, Pneumococcal, Meningococcal, H. influenzae vaccines
• Prevents infections in transfusion recipients or splenectomized patients

---

🔹 G. Psychosocial Support

• Counseling for child and family
• Support groups and educational guidance

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🧬 Advanced Therapies

🔹 H. Bone Marrow or Stem Cell Transplantation

• Only curative treatment
• Best results when performed in early childhood with matched sibling donor
• Requires lifelong follow-up post-transplant

---

🔹 I. Gene Therapy (Emerging)

• Aims to correct the defective gene
• Still under clinical trials in many countries

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📌 Summary Table: Medical Management

Type	Key Management
Minor	Observation, folic acid, genetic counseling
Intermedia	Occasional transfusions, hydroxyurea, chelation if needed
Major	Regular transfusions, iron chelation, endocrine care, stem cell transplant, gene therapy (future)

🏥 Surgical Management of Thalassemia

Surgical management is not a primary treatment but may be necessary in specific situations, especially in thalassemia major or thalassemia intermedia to manage complications or provide curative therapy.

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✅ 1. Splenectomy (Surgical Removal of the Spleen)

📌 Indications:

• Massive splenomegaly causing pain or discomfort
• Hypersplenism (overactive spleen destroying healthy RBCs and platelets)
• Increased transfusion requirement due to spleen destruction of transfused RBCs
• Low platelet or WBC count due to splenic pooling

⚠️ Precautions Before Surgery:

• Ensure Hb > 10 g/dL via transfusion
• Preoperative vaccinations:
  • Pneumococcal
  • Haemophilus influenzae type B
  • Meningococcal
• Prophylactic antibiotics

🏥 Post-operative Care:

• Monitor for bleeding, infection, and respiratory complications
• Long-term infection risk → patient education on signs of sepsis
• Lifelong prophylactic antibiotics may be needed in children
• Educate on fever monitoring and early medical consultation

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✅ 2. Bone Marrow or Hematopoietic Stem Cell Transplantation (HSCT)

📌 Only Curative Option for Thalassemia Major

✅ Indications:

• Severe beta thalassemia major
• Age usually <14 years
• Matched sibling donor (HLA-compatible)

🧬 Procedure:

• High-dose chemotherapy (to destroy defective marrow)
• Transplantation of healthy donor stem cells
• New cells produce normal hemoglobin

💉 Risks and Considerations:

• Graft-versus-host disease (GVHD)
• Infection risk due to immunosuppression
• Expensive and resource-intensive
• Close follow-up required for years

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✅ 3. Cholecystectomy (Gallbladder Removal)

📌 Indications:

• Repeated gallstone attacks due to chronic hemolysis (↑ bilirubin)
• Performed laparoscopically or during splenectomy if needed

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✅ 4. Orthopedic or Dental Surgeries

Often needed in long-standing cases with bone deformities

• Corrective surgeries for facial/jaw deformities
• Dental surgeries may be required for malocclusion caused by marrow expansion
• Transfusion support and factor coverage required pre- and post-operatively

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📌 Summary Table: Surgical Management

Procedure	Indication	Notes
Splenectomy	Hypersplenism, increased transfusion needs	Needs pre-op vaccines + antibiotics
Bone marrow transplant	Curative in thalassemia major	Best in young patients with HLA match
Cholecystectomy	Gallstones due to hemolysis	May be done with splenectomy
Corrective surgeries	Skeletal/facial deformities	Rare, cosmetic/functional indication

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👩‍⚕️ Nursing Considerations for Surgical Patients with Thalassemia

Task	Nursing Role
Pre-op preparation	Ensure vaccination, transfusions, educate patient
Intra-op monitoring	Observe for bleeding (coagulopathy risk)
Post-op care	Pain management, wound care, infection surveillance
Education	Infection control post-splenectomy, medication adherence
Long-term follow-up	Especially for transplant patients – watch for GVHD and relapse

👩‍⚕️ Nursing Management of Thalassemia

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🧠 I. Nursing Objectives

• Maintain adequate oxygenation and prevent complications of anemia
• Support safe transfusion therapy
• Prevent and manage iron overload
• Promote growth, nutrition, and psychosocial well-being
• Educate patient and family about disease, self-care, and long-term management

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📋 II. General Nursing Interventions

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1️⃣ Assessment

What to Monitor	Why
Vital signs	Detect anemia-related tachycardia, hypotension, fever
Hemoglobin levels	Assess severity of anemia and need for transfusion
Serum ferritin, liver enzymes	Monitor for iron overload
Growth parameters	Assess for growth retardation in children
Spleen and liver size	Detect hepatosplenomegaly
Signs of infection	Post-splenectomy patients are at high risk

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2️⃣ Transfusion Care

Task	Nursing Action
Pre-transfusion	Verify blood type, check vital signs, educate patient
During transfusion	Monitor for reactions (rash, fever, chills, dyspnea)
Post-transfusion	Document response, observe for delayed reactions
Use leukocyte-reduced and cross-matched packed RBCs	Reduces risk of alloimmunization and reaction

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3️⃣ Iron Chelation Therapy Support

Drug	Nursing Responsibilities
Deferoxamine (SC/IV)	Teach infusion pump use, rotate sites
Deferasirox / Deferiprone (oral)	Monitor for GI upset, neutropenia, liver function
Lab monitoring	Check serum ferritin and iron studies regularly

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4️⃣ Nutritional Care

Goal	Action
Prevent malnutrition	Encourage balanced diet with high-protein, iron-free foods
Folic acid supplementation	Support RBC formation
Avoid iron-rich foods or supplements	Iron already elevated from transfusions

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5️⃣ Psychosocial & Developmental Support

Age Group	Nursing Role
Children	Encourage schooling, play therapy, support physical growth
Adolescents	Support coping with body image, fertility counseling
Family	Involve in decision-making, provide emotional support
All ages	Refer to support groups, genetic counseling if needed

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6️⃣ Infection Prevention (esp. post-splenectomy)

• Monitor for fever and signs of sepsis
• Educate about urgent medical care for any fever
• Ensure vaccinations (pneumococcal, meningococcal, Hib, hepatitis B)
• Support prophylactic antibiotics if prescribed

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📚 III. Patient & Family Education

Topic	Education Point
Disease understanding	Nature of inherited anemia, importance of regular care
Transfusion therapy	Importance of timely transfusions, signs of reactions
Iron overload	Why chelation is needed, drug administration techniques
Infection prevention	Hygiene, vaccines, fever awareness
Fertility & pregnancy	Genetic counseling before planning children
Medication compliance	Importance of taking folic acid, chelators, and follow-up labs
Lifestyle	Avoid strenuous activity during low Hb, no smoking/alcohol

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✅ IV. Evaluation Criteria

• Stable hemoglobin levels maintained
• No signs of iron overload or transfusion reaction
• Normal growth in children
• Compliance with medication and transfusion schedule
• Family understands disease and management plan
• Patient demonstrates improved coping and self-care

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📌 Key Nursing Considerations

✅ Never give iron supplements to thalassemia patients unless iron-deficiency is proven
✅ Use strict transfusion protocols to reduce risk of reaction
✅ Monitor for iron toxicity symptoms: fatigue, joint pain, liver/heart issues
✅ Always observe for psychological distress in chronic transfusion patients
✅ Encourage lifelong follow-up and specialist care access

⚠️ Complications of Thalassemia

Complications depend on the type and severity, especially in Thalassemia Major due to chronic anemia, repeated transfusions, and iron overload.

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🧠 I. Major Complications

🩸 1. Severe Anemia

• Fatigue, pallor, weakness
• Poor school performance in children
• May lead to cardiac failure if untreated

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🧠 2. Iron Overload (Hemosiderosis)

Occurs due to repeated blood transfusions and increased iron absorption

Affected Organ	Resulting Complication
Heart	Cardiomyopathy, arrhythmias, heart failure
Liver	Hepatomegaly, fibrosis, cirrhosis
Endocrine glands	Diabetes mellitus, hypothyroidism, delayed puberty, infertility
Skin	Bronze discoloration

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🦴 3. Bone Deformities

• Marrow expansion due to chronic erythropoiesis
• Frontal bossing, prominent cheekbones, dental malocclusion
• Osteoporosis and increased fracture risk

---

🧒 4. Growth and Developmental Delays

• Short stature, delayed puberty
• Poor weight gain
• Emotional and cognitive impact

---

💉 5. Transfusion-Related Complications

• Allergic reactions, febrile reactions
• Transmission of infections: Hepatitis B/C, HIV
• Alloimmunization: Body forms antibodies against donor RBCs → reduces transfusion effectiveness

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🦠 6. Increased Risk of Infection

• Especially after splenectomy
• Life-threatening infections can occur without prompt treatment

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💔 7. Psychosocial Issues

• Chronic illness → anxiety, depression
• Social withdrawal, absenteeism, reduced quality of life

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📌 II. Key Points to Remember (Nursing & Exam Focus)

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🔹 Understanding the Disease

✅ Thalassemia is a genetic disorder of hemoglobin synthesis
✅ Can affect alpha or beta chains → causes ineffective erythropoiesis and hemolysis

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🔹 Classification

✅ Thalassemia types:

• Minor: Mild, often asymptomatic
• Intermedia: Moderate anemia, variable symptoms
• Major: Severe, transfusion-dependent anemia

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🔹 Management Essentials

✅ Regular blood transfusions maintain Hb >9 g/dL in major cases
✅ Iron chelation is critical to prevent organ damage from iron overload
✅ Folic acid supplementation supports RBC production
✅ Bone marrow transplant is the only cure (if eligible)

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🔹 Infection Control

✅ Splenectomy increases infection risk → vaccination + antibiotics
✅ Prompt treatment of any fever is vital

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🔹 Psychosocial Support

✅ Chronic care requires emotional and social support
✅ Family education and genetic counseling are key parts of care

---

🔹 Nursing Role

✅ Monitor for anemia, growth delay, iron overload signs
✅ Educate about medication, transfusions, infection prevention
✅ Provide holistic care including emotional and developmental support

🧬 Leukemias

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📘 Definition

Leukemia is a group of malignant disorders of the blood-forming tissues, primarily the bone marrow and lymphatic system, characterized by:

• Uncontrolled proliferation of abnormal white blood cells (WBCs)
• These abnormal WBCs do not function properly, crowding out healthy blood cells
• Results in anemia, immunosuppression, and bleeding tendencies

Leukemia can be acute (rapid onset) or chronic (slow progression), and involve lymphoid or myeloid cell lines.

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❓ Causes of Leukemia

Leukemia has no single known cause, but several risk factors are associated:

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🔹 1. Genetic Factors

• Chromosomal abnormalities (e.g., Philadelphia chromosome in CML)
• Inherited syndromes:
  • Down syndrome
  • Fanconi anemia
  • Bloom syndrome

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🔹 2. Environmental Factors

• Exposure to ionizing radiation (atomic bomb survivors, radiation therapy)
• Exposure to chemicals:
  • Benzene
  • Pesticides
  • Certain industrial solvents

---

🔹 3. Viral Infections

• Human T-lymphotropic virus (HTLV-1)
• Epstein-Barr virus (EBV) – linked to Burkitt’s lymphoma/leukemia

---

🔹 4. Previous Cancer Treatment

• Chemotherapy (especially alkylating agents)
• Radiation therapy

---

🔹 5. Immune System Disorders

• Autoimmune diseases
• Immunodeficiency states (e.g., HIV/AIDS)

---

🔹 6. Smoking and Lifestyle

• Smoking is linked with acute myeloid leukemia (AML)
• Obesity may increase the risk of certain leukemias

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🔹 7. Unknown/Idiopathic

• In many cases, especially in children, the exact cause remains unknown

🔠 Types of Leukemia.

Leukemias are classified based on:

1. Speed of progression:
   • Acute – rapid onset
   • Chronic – slow, gradual progression
2. Type of white blood cell affected:
   • Lymphoid (affecting lymphocytes)
   • Myeloid (affecting granulocytes, monocytes)

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📊 Classification Table of Leukemias

Type	Full Name	Progression	Affected Cell Line	Common in
ALL	Acute Lymphoblastic Leukemia	Rapid	Lymphoid precursors	Children (peak: 2–5 yrs)
AML	Acute Myeloid Leukemia	Rapid	Myeloid precursors	Adults >40 yrs
CLL	Chronic Lymphocytic Leukemia	Slow	Mature lymphocytes	Elderly (over 60 yrs)
CML	Chronic Myeloid Leukemia	Slow → can accelerate	Granulocytes	Adults (30–60 yrs)

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🧒 1. Acute Lymphoblastic Leukemia (ALL)

📘 Description:

• Malignancy of immature lymphoid cells (lymphoblasts)
• Commonest cancer in children
• Responds well to chemotherapy

⚠️ Key Features:

• Sudden onset: fever, fatigue, bone pain, bleeding
• Anemia, infections, enlarged liver/spleen/lymph nodes
• CNS involvement (headache, vomiting, nerve palsies)

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👨‍🦱 2. Acute Myeloid Leukemia (AML)

📘 Description:

• Malignancy of immature myeloid cells (myeloblasts)
• Affects mostly adults but can occur at any age
• May follow previous chemotherapy or radiation

⚠️ Key Features:

• Rapid onset: fatigue, pallor, frequent infections
• Bleeding (gum, nose, petechiae)
• Bone pain, splenomegaly
• Auer rods seen in blood smear (specific for AML)

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👴 3. Chronic Lymphocytic Leukemia (CLL)

📘 Description:

• Slow-growing cancer of mature B-lymphocytes
• Most common chronic leukemia in elderly
• Often discovered incidentally on routine blood tests

⚠️ Key Features:

• Fatigue, weight loss, recurrent infections
• Painless lymphadenopathy
• Splenomegaly, anemia
• May remain asymptomatic for years

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🧔 4. Chronic Myeloid Leukemia (CML)

📘 Description:

• Uncontrolled production of granulocytes (neutrophils, eosinophils, basophils)
• Associated with Philadelphia chromosome (t[9;22])
• May progress to blast crisis (resembles acute leukemia)

⚠️ Key Features:

• Fatigue, weight loss, night sweats
• Fullness in left abdomen (splenomegaly)
• Increased WBC count in CBC
• May be diagnosed during a routine health check

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📌 Summary Table

Feature	ALL	AML	CLL	CML
Onset	Sudden	Sudden	Slow	Slow
Age group	Children	Adults	Elderly	Adults (30–60 yrs)
Cell type	Lymphoblasts	Myeloblasts	Mature B cells	Granulocytes
Common signs	Bone pain, CNS signs	Bleeding, fatigue	Lymphadenopathy	Splenomegaly
Lab findings	↑ lymphoblasts	↑ myeloblasts + Auer rods	↑ mature lymphocytes	↑ granulocytes; Philadelphia chromosome
Prognosis	Good in children	Variable	Chronic, may live years	Chronic phase → blast crisis

🧬 Pathophysiology of Leukemia.

Leukemia involves the uncontrolled proliferation of abnormal white blood cells in the bone marrow. These abnormal cells crowd out normal cells, resulting in:

• ↓ RBCs → anemia
• ↓ Platelets → bleeding
• ↓ Normal WBCs → infections

Each type of leukemia affects different cell lines and progresses differently.

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1️⃣ Acute Lymphoblastic Leukemia (ALL)

🧬 Pathogenesis:

1. Mutation occurs in lymphoid stem cells (mostly B-lymphoblasts)
2. Leads to overproduction of immature lymphoblasts
3. These abnormal cells fail to mature and enter the bloodstream
4. They infiltrate:
   • Bone marrow → ↓ RBCs, WBCs, and platelets
   • Lymph nodes, spleen, liver
   • CNS and meninges (in advanced cases)

🔁 Result:

• Pancytopenia (due to marrow crowding)
• Bone pain, CNS symptoms, infections, bleeding

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2️⃣ Acute Myeloid Leukemia (AML)

🧬 Pathogenesis:

1. Mutation in myeloid precursor cells
2. Accumulation of immature myeloblasts in bone marrow
3. Disruption of normal hematopoiesis:
   • ↓ RBCs → anemia
   • ↓ Platelets → bleeding
   • ↓ Mature WBCs → infections
4. Myeloblasts enter blood, liver, spleen, and may infiltrate gums, skin

🔬 Special Feature:

• Auer rods seen in myeloblasts on blood smear

🔁 Result:

• Rapid onset of fatigue, infections, gum bleeding, petechiae

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3️⃣ Chronic Lymphocytic Leukemia (CLL)

🧬 Pathogenesis:

1. Malignancy of mature B-lymphocytes
2. These B-cells are morphologically mature but functionally incompetent
3. Accumulate in bone marrow, blood, lymph nodes
4. Slow disease progression; cells live longer but do not function
5. Suppression of normal immune function → hypogammaglobulinemia

🔁 Result:

• Immunosuppression, lymphadenopathy, splenomegaly, fatigue

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4️⃣ Chronic Myeloid Leukemia (CML)

🧬 Pathogenesis:

1. Occurs due to Philadelphia chromosome (translocation t[9;22])
2. Forms BCR-ABL fusion gene → codes for abnormal tyrosine kinase
3. This enzyme causes:
   • Uncontrolled proliferation of granulocytic WBCs (neutrophils, eosinophils, basophils)
   • Suppression of apoptosis (cell death)

Disease Progresses in 3 Phases:

• Chronic phase → slow progression, minimal symptoms
• Accelerated phase → increased WBCs, worsening symptoms
• Blast crisis → resembles acute leukemia, poor prognosis

🔁 Result:

• Very high WBC count, splenomegaly, fatigue, weight loss

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🔁 General Pathophysiological Effects in All Leukemias

System Affected	Changes
Bone Marrow	Crowding by leukemic cells → ↓ RBCs, ↓ platelets, ↓ normal WBCs
Immune system	Poor immunity due to abnormal or absent lymphocytes
Circulation	Risk of bleeding, infections, anemia
Organs	Infiltration of liver, spleen, CNS, lymph nodes
Metabolism	↑ cell turnover → hyperuricemia, ↑ LDH

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📌 Summary Table: Pathophysiology by Type

Type	Origin	Key Events	Unique Features
ALL	Lymphoid progenitors	Lymphoblast accumulation	CNS involvement common
AML	Myeloid progenitors	Myeloblast crowding	Auer rods, gum/skin infiltration
CLL	Mature B-lymphocytes	Ineffective immunity, long-living abnormal cells	Hypogammaglobulinemia
CML	Granulocytes	BCR-ABL fusion gene → excessive granulocytes	Triphasic progression, high WBC

⚠️ Signs and Symptoms of Leukemia

Leukemia symptoms vary depending on the type (acute vs chronic) and cell lineage (lymphoid vs myeloid). However, all types share common features due to bone marrow suppression and organ infiltration.

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🧠 I. General Signs & Symptoms (All Types)

Affected Cell Line	Clinical Effects
↓ RBCs	Fatigue, pallor, weakness (anemia)
↓ Platelets	Easy bruising, bleeding gums, petechiae, nosebleeds
↓ Functional WBCs	Frequent infections, fever
Bone marrow overactivity	Bone/joint pain, especially in children
Organ infiltration	Enlarged liver, spleen, lymph nodes

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🔹 Type-Specific Signs & Symptoms

✅ 1. Acute Lymphoblastic Leukemia (ALL)

Common in children

Symptom	Explanation
Fatigue, pallor	Due to anemia
Bone pain, joint swelling	Marrow expansion
Recurrent infections	Low functional WBCs
Bruising, gum/nosebleeds	Thrombocytopenia
CNS involvement	Headache, vomiting, vision problems
Fever, night sweats	Common constitutional symptoms
Lymphadenopathy, hepatosplenomegaly	Leukemic cell infiltration

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✅ 2. Acute Myeloid Leukemia (AML)

More common in adults

Symptom	Explanation
Sudden fatigue, weakness	Severe anemia
Gum hypertrophy and bleeding	Infiltration by myeloblasts
Petechiae, ecchymosis	Low platelets
Fever, infections	Neutropenia
Bone tenderness	Marrow crowding
Possible DIC (especially M3 subtype)	Clotting abnormalities

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✅ 3. Chronic Lymphocytic Leukemia (CLL)

Affects elderly, often asymptomatic at first

Symptom	Explanation
Painless lymph node enlargement	Classic early sign
Fatigue	Due to anemia
Recurrent infections	Hypogammaglobulinemia
Weight loss, fever, night sweats	Late stage (B symptoms)
Splenomegaly, hepatomegaly	Due to cell infiltration

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✅ 4. Chronic Myeloid Leukemia (CML)

Affects middle-aged adults

Symptom	Explanation
Early: asymptomatic	Often found on routine blood test
Fatigue, night sweats	Due to high WBC turnover
Left-sided abdominal fullness	Massive splenomegaly
Weight loss, anemia	Disease progression
Bleeding episodes	Due to thrombocytopenia
Blast crisis phase	Resembles acute leukemia (rapid deterioration)

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🔬 II. Diagnostic Investigations

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🧪 1. Blood Tests

Test	Findings
CBC (Complete Blood Count)	↓ RBCs, ↓ platelets, ↑ or ↓ WBCs
Peripheral blood smear	Blast cells (ALL, AML), mature lymphocytes (CLL), granulocytosis (CML)
Reticulocyte count	Usually ↓ due to marrow suppression

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🔬 2. Bone Marrow Aspiration & Biopsy

Gold standard test for diagnosis

Findings	Interpretation
>20% blasts in marrow	Diagnostic for acute leukemia (ALL/AML)
Abnormal mature lymphocytes	Suggestive of CLL
Increased granulocytes and basophils	Suggestive of CML

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🧬 3. Cytogenetic and Molecular Studies

Test	Use
Philadelphia chromosome (t[9;22])	Diagnostic for CML
Flow cytometry	Classifies leukemia type by identifying cell surface markers
PCR for BCR-ABL gene	CML confirmation
Karyotyping	Detects chromosomal abnormalities (esp. in AML & ALL)

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🧪 4. Additional Investigations

Test	Purpose
Liver and kidney function tests	Pre-chemotherapy assessment
Coagulation profile (PT, aPTT, D-dimer)	For DIC risk (esp. AML M3)
Chest X-ray, CT scan	To detect mediastinal mass or lymphadenopathy
Lumbar puncture (CSF analysis)	If CNS leukemia suspected (mostly in ALL)
LDH & Uric acid	Elevated due to high cell turnover

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📌 Summary Table: Diagnostic Clues by Type

Type	Key Diagnostic Features
ALL	↑ Lymphoblasts, bone pain, CNS symptoms, common in kids
AML	↑ Myeloblasts, Auer rods, bleeding, gum involvement
CLL	↑ Mature lymphocytes, lymphadenopathy, elderly patients
CML	↑ Granulocytes, splenomegaly, Philadelphia chromosome

💊 Medical Management of Leukemia (ALL, AML, CLL, CML)

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🧬 1. Acute Lymphoblastic Leukemia (ALL)

✅ Goals:

• Eradicate leukemic blasts
• Restore normal hematopoiesis
• Prevent CNS involvement
• Maintain remission

💊 Phases of Treatment:

Phase	Treatment
Induction	High-dose chemotherapy (e.g., vincristine, daunorubicin, prednisone, asparaginase) → aim for remission
Consolidation/Intensification	Continued chemotherapy to eliminate hidden leukemic cells
Maintenance	Lower-dose oral chemotherapy for 2–3 years (e.g., methotrexate, 6-mercaptopurine)
CNS Prophylaxis	Intrathecal chemotherapy (methotrexate) ± cranial irradiation
Supportive Care	Blood transfusions, antibiotics, antifungals, nutritional support
Targeted therapy (Ph+ ALL)	Tyrosine kinase inhibitors (Imatinib)

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🧬 2. Acute Myeloid Leukemia (AML)

✅ Goals:

• Achieve complete remission
• Prevent relapse
• Support during neutropenic periods

💊 Phases of Treatment:

Phase	Treatment
Induction	“7+3” regimen: cytarabine (7 days) + anthracycline (3 days) like daunorubicin
Consolidation	High-dose cytarabine to destroy residual disease
Stem Cell Transplant	For high-risk or relapsed cases
Targeted therapy	FLT3 inhibitors (Midostaurin) or IDH1/2 inhibitors (for mutation-positive AML)
Supportive Care	Platelet and RBC transfusions, antibiotics, antifungals, growth factors (G-CSF)
Differentiation therapy (M3 subtype/APL)	All-trans-retinoic acid (ATRA) + arsenic trioxide

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🧬 3. Chronic Lymphocytic Leukemia (CLL)

✅ Goals:

• Control disease progression
• Improve quality of life
• Delay symptoms

💊 Treatment Approach:

Stage	Management
Early stage (asymptomatic)	Watchful waiting (no immediate treatment)
Symptomatic or advanced disease

• Chemotherapy: Fludarabine + cyclophosphamide
• Targeted therapy:
  • BTK inhibitors: Ibrutinib, Acalabrutinib
  • BCL-2 inhibitors: Venetoclax
• Monoclonal antibodies: Rituximab (anti-CD20)
  | Supportive Care | Antibiotics, antivirals, IVIG for infections, allopurinol for uric acid control |

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🧬 4. Chronic Myeloid Leukemia (CML)

✅ Goals:

• Suppress BCR-ABL gene activity
• Normalize blood counts
• Delay or prevent blast crisis

💊 Mainstay of Treatment:

Drug Type	Example	Mechanism
Tyrosine Kinase Inhibitors (TKIs)

• Imatinib (first-line)
• Dasatinib, Nilotinib, Bosutinib
  | Block BCR-ABL fusion protein responsible for uncontrolled cell growth |

💉 Other Interventions:

• Hydroxyurea: Used to reduce WBC count initially
• Allogeneic stem cell transplant: For resistant or blast crisis phase
• Supportive therapy: Treat anemia, bleeding, infections

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🩸 Supportive Therapies for All Leukemia Types

Therapy	Purpose
Blood transfusions	Treat anemia and bleeding risk
Platelet transfusions	When platelets <10,000/mm³ or active bleeding
Antibiotics/antifungals/antivirals	During neutropenia to prevent infections
Growth factors (e.g., G-CSF)	Boost WBC production post-chemotherapy
Nutritional support	High-protein, neutropenic diet during therapy
Psychological counseling	Support for patients and families

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📌 Summary Table

Type	First-Line Treatment	Targeted/Additional
ALL	Multi-drug chemo + CNS prophylaxis	Imatinib if Ph+, stem cell transplant (high-risk)
AML	Cytarabine + daunorubicin	FLT3 inhibitors, ATRA (APL subtype), transplant
CLL	Observation → chemo/targeted	Ibrutinib, Venetoclax, Rituximab
CML	Imatinib (TKI)	Nilotinib, Dasatinib, stem cell transplant

🏥 Surgical Management of Leukemia

Surgical treatment is not curative on its own but may be required as part of advanced or supportive therapy, especially in cases like resistant or relapsed leukemia, or for complication management.

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✅ 1. Hematopoietic Stem Cell Transplantation (HSCT)

Also known as Bone Marrow Transplant (BMT)

📘 Definition:

A procedure in which damaged bone marrow is replaced with healthy hematopoietic stem cells, either from the patient (autologous) or a donor (allogeneic).

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📌 Indications in Leukemia:

Type of Leukemia	Indication for Transplant
ALL (Acute Lymphoblastic Leukemia)	High-risk or relapsed cases
AML (Acute Myeloid Leukemia)	Poor cytogenetics, relapse, or non-response to chemo
CML (Chronic Myeloid Leukemia)	TKI-resistant or blast crisis phase
CLL (Chronic Lymphocytic Leukemia)	Rarely indicated; only in aggressive, refractory cases

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🔄 Types of Transplant:

Type	Source of Stem Cells	Used When
Autologous	From the patient	In remission; less common in leukemia
Allogeneic	From a matched donor (sibling/unrelated)	Most effective, especially for ALL, AML

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🛑 Pre-transplant Procedure:

• Conditioning regimen with high-dose chemotherapy or radiation to destroy diseased marrow
• Prevent rejection and relapse

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🧩 Post-transplant Care:

• Monitor for Graft vs Host Disease (GVHD)
• Administer immunosuppressants (e.g., cyclosporine, tacrolimus)
• Infection control: strict neutropenic precautions
• Long-term monitoring for relapse and organ function

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✅ 2. Splenectomy (Rarely in Leukemia)

Surgical removal of the spleen is not common, but may be considered in specific cases:

📌 Indications in Leukemia:

• Massive splenomegaly causing pain or discomfort
• Hypersplenism (destruction of blood cells) not responsive to medical therapy
• Supportive in hairy cell leukemia or CLL with spleen involvement

⚠️ Risks & Considerations:

• High risk of infection post-splenectomy → vaccination required
• Lifelong risk of overwhelming post-splenectomy infection (OPSI)

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✅ 3. Central Line or Port Placement

• Needed for chemotherapy administration, frequent blood sampling, or stem cell infusion
• Requires sterile technique, and nurse education on central line care

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📋 Surgical Management Summary Table

Procedure	Purpose	Indication
Stem Cell Transplant	Curative or rescue therapy	High-risk/relapsed leukemia
Splenectomy	Reduce blood cell destruction	CLL or hairy cell leukemia with massive spleen
Central Line Insertion	Long-term IV access	Chemotherapy, transfusions

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👩‍⚕️ Nursing Considerations (Surgical Care)

Stage	Nursing Role
Pre-op (transplant/splenectomy)	Explain procedure, obtain consent, pre-op labs, administer vaccines
Post-op monitoring	Observe for bleeding, infection, vital signs, wound care
Infection prevention	Hand hygiene, neutropenic precautions, oral care
Psychosocial support	Address anxiety, provide emotional and family support
Educate patient/family	Central line care, signs of GVHD or rejection, long-term precautions

👩‍⚕️ Nursing Management of Leukemia

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🧠 I. Nursing Objectives

1. Manage effects of anemia, bleeding, and infections
2. Provide safe chemotherapy and transfusion care
3. Prevent complications (GVHD, mucositis, sepsis)
4. Support nutritional and emotional needs
5. Educate patient and family for home care and follow-up

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📋 II. General Nursing Interventions

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1️⃣ Assessment & Monitoring

What to Monitor	Why
Vital signs	Detect fever, tachycardia, hypotension (signs of infection or bleeding)
CBC & differential	Monitor WBCs, Hb, platelets
Signs of anemia	Pallor, fatigue, breathlessness
Signs of infection	Fever, cough, sore throat, pus
Signs of bleeding	Petechiae, bruises, gum/nose bleeding, hematuria
Chemotherapy side effects	Nausea, mucositis, alopecia, myelosuppression
Bone pain or swelling	May indicate disease progression or infiltration

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2️⃣ Infection Prevention (Especially in Neutropenia)

Intervention	Action
Isolation precautions	Reverse isolation if ANC < 500/mm³
Hand hygiene	Strict for staff, patient, and visitors
No fresh flowers/fruits	Risk of fungal/bacterial exposure
Oral care	Chlorhexidine or saline rinse to prevent mucositis/infection
Monitor central lines	Prevent catheter-related infections
Administer antibiotics/antifungals	As prescribed during febrile neutropenia

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3️⃣ Bleeding Precautions (In Thrombocytopenia)

Precaution	Purpose
Avoid IM injections	Prevent hematoma formation
Use soft toothbrush & electric razor	Reduce mucosal/skin trauma
Apply pressure post-venipuncture	Prevent bleeding
Monitor for signs of internal bleeding	Hematuria, melena, dizziness
Transfuse platelets as ordered	Usually <10,000–20,000/mm³ or active bleeding

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4️⃣ Anemia Management

• Encourage rest, oxygen therapy, and cluster nursing activities
• Provide packed RBC transfusions as ordered
• Promote high-iron, high-protein diet (if appropriate)
• Monitor for fatigue and dyspnea on exertion

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5️⃣ Chemotherapy & Transfusion Care

Task	Nursing Role
Pre-chemo	Check lab results, hydrate patient, pre-medicate (antiemetics)
During chemo	Monitor IV site, infusion reactions, vitals
Post-chemo	Observe for neutropenia, mucositis, nausea, alopecia
Blood transfusions	Ensure proper ID, monitor for reactions (fever, chills, back pain, rash)

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6️⃣ Nutritional Support

• High-calorie, high-protein diet
• Small, frequent meals
• Neutropenic diet if ANC very low (no raw fruits/veg or undercooked meats)
• IV fluids or TPN if mucositis prevents oral intake

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7️⃣ Psychosocial and Emotional Support

Intervention	Notes
Encourage expression of fears	Allow verbalization of anxiety
Provide support groups	Peer support helps emotional coping
Involve family	Family-centered care improves outcomes
Support body image issues	Especially in adolescents (due to alopecia, weight changes)

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📚 III. Patient & Family Education

Topic	Education Content
Nature of disease	Acute vs. chronic, need for long-term care
Infection signs	Fever >100.4°F, sore throat, wound redness
Bleeding signs	Bruising, gum bleeding, blood in stool/urine
Medication adherence	Chemotherapy, antibiotics, growth factors
Nutrition & hygiene	Safe foods, oral care, hydration
Follow-up care	Regular labs, post-chemo monitoring
Emotional wellbeing	Coping mechanisms, stress relief

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✅ IV. Evaluation Criteria

• Patient is free from infection and bleeding
• Maintains adequate oxygenation and nutrition
• Demonstrates understanding of home care and medication
• Participates in treatment and decision-making
• Verbalizes reduced anxiety and improved coping

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📌 Key Nursing Points

✅ Always assume neutropenic precautions during low WBC phase
✅ Monitor for tumor lysis syndrome (hyperkalemia, hyperuricemia) in acute leukemia
✅ Ensure patient has emergency contact and knows when to report symptoms
✅ Be alert for GVHD signs after stem cell transplant (skin rash, diarrhea, jaundice)
✅ Educate family on importance of vaccinations, hygiene, and follow-up

⚠️ Complications of Leukemia

Leukemia and its treatments can lead to life-threatening complications. Early detection and preventive nursing care are essential.

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🧠 I. Disease-Related Complications

Complication	Description
Anemia	↓ RBCs → fatigue, weakness, breathlessness
Thrombocytopenia	↓ Platelets → easy bruising, bleeding gums, petechiae, internal bleeding
Neutropenia	↓ WBCs → ↑ susceptibility to infections
Bone pain and fractures	Due to marrow expansion (especially in ALL)
Organ infiltration	Leukemic cells infiltrate liver, spleen, lymph nodes, CNS
CNS involvement (esp. in ALL)	Headache, seizures, cranial nerve palsies
Tumor lysis syndrome (TLS)	After chemo → ↑ uric acid, K⁺, phosphate → renal failure, arrhythmias

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💉 II. Treatment-Related Complications

🔹 Chemotherapy

• Myelosuppression → neutropenia, anemia, thrombocytopenia
• Mucositis → mouth ulcers, painful swallowing
• Nausea, vomiting, alopecia
• Hepatic/renal toxicity
• Infertility in some cases

🔹 Radiation Therapy

• Fatigue, skin burns, secondary cancers (long-term)

🔹 Stem Cell Transplant

• Graft-versus-host disease (GVHD): skin rash, diarrhea, jaundice
• Infection risk due to immunosuppression

🔹 Transfusions

• Allergic or febrile reactions
• Iron overload from repeated transfusions
• Transmission of infections (e.g., Hepatitis B/C, HIV — rare due to screening)

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🧩 Chronic Leukemia-Specific Complications

Condition	Complications
CLL	Recurrent infections, autoimmune hemolytic anemia, Richter’s transformation (into aggressive lymphoma)
CML	Blast crisis (sudden transformation to acute leukemia), resistance to TKIs

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📌 Key Points to Remember

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🔹 Understanding the Disease

✅ Leukemia = uncontrolled WBC production
✅ Can be acute (fast-growing) or chronic (slow-growing)
✅ Affects lymphoid (ALL, CLL) or myeloid (AML, CML) cell lines

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🔹 Early Symptoms to Recognize

✅ Fatigue, fever, bleeding, bone pain, recurrent infections
✅ In ALL: CNS symptoms (headache, vomiting)
✅ In CML: splenomegaly, night sweats, very high WBCs

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🔹 Diagnostic Highlights

✅ CBC shows ↑ or ↓ WBCs, ↓ Hb, ↓ platelets
✅ Bone marrow aspiration confirms diagnosis
✅ Philadelphia chromosome = diagnostic for CML
✅ Flow cytometry helps subtype leukemias

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🔹 Treatment Principles

✅ ALL/AML: chemotherapy + CNS prophylaxis + transplant (in selected)
✅ CLL: may not need early treatment; targeted therapy in symptomatic cases
✅ CML: managed with tyrosine kinase inhibitors (Imatinib)

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🔹 Nursing Priorities

✅ Infection control during neutropenia
✅ Bleeding precautions during thrombocytopenia
✅ Emotional support and family involvement
✅ Monitor for tumor lysis syndrome, transfusion reactions
✅ Educate on medications, follow-ups, and hygiene practices

🧬 Leukopenia

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📘 Definition

Leukopenia is a condition in which the total white blood cell (WBC) count falls below 4,000 cells/mm³, leading to increased susceptibility to infections. It mainly affects neutrophils, the body’s first line of defense against bacteria.

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❓ Causes of Leukopenia

Category	Specific Causes
Infections	Viral (HIV, hepatitis, influenza, measles), tuberculosis
Autoimmune disorders	Lupus (SLE), rheumatoid arthritis
Bone marrow suppression	Aplastic anemia, leukemia, chemotherapy, radiation
Drugs & toxins	Chemotherapy, antipsychotics, antibiotics (e.g., chloramphenicol), antiepileptics
Nutritional deficiencies	Vitamin B12 or folate deficiency
Hypersplenism	Excessive sequestration of WBCs in the spleen
Congenital disorders	Kostmann syndrome (congenital neutropenia)
Endocrine disorders	Hypothyroidism, adrenal insufficiency

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🔠 Types of Leukopenia (Based on Affected WBC Type)

Type	Definition
Neutropenia	↓ Neutrophils (most common and dangerous form)
Lymphocytopenia	↓ Lymphocytes (affects viral defense and immunity)
Monocytopenia	↓ Monocytes (rare)
Eosinopenia / Basopenia	Rare, often clinically insignificant

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🧬 Pathophysiology

1. Impaired production of WBCs → e.g., bone marrow failure, chemotherapy
2. Increased destruction of WBCs → autoimmune diseases, infections
3. Sequestration in spleen → hypersplenism
4. Result: ↓ circulating WBCs → weak immune response → ↑ infection risk

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⚠️ Signs and Symptoms

System	Common Symptoms
General	Fever, chills, malaise, fatigue
Respiratory	Sore throat, cough, shortness of breath
Skin	Non-healing wounds, abscesses, ulcers, boils
GI/Urinary	Diarrhea, burning urination
Oral	Mouth ulcers, gum infections, candidiasis
Severe Neutropenia (<500 cells/mm³)	Risk of life-threatening infections without obvious symptoms

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🔬 Diagnosis

Test	Purpose
CBC with differential	Confirms ↓ total WBC and which type is reduced
Peripheral smear	Visualizes abnormal cells or infections
Bone marrow aspiration/biopsy	Assesses marrow function (hypoplasia, infiltration)
Infection screening	Blood, urine, sputum cultures
Vitamin B12, folate levels	Rule out nutritional causes
HIV, Hepatitis serology	If viral cause suspected
Autoimmune screening	ANA, RF for lupus or RA
Genetic testing	In congenital neutropenia

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💊 Medical Management

Cause	Treatment
Infection-induced	Treat underlying infection, broad-spectrum antibiotics
Chemotherapy-induced	Adjust chemo dose, delay therapy if needed
Nutritional	Vitamin B12 or folate supplementation
Autoimmune	Corticosteroids, immunosuppressants
Bone marrow failure	Stem cell transplant (if severe), supportive care
Drug-induced	Stop causative drug

🔹 Supportive Therapy:

• Granulocyte Colony-Stimulating Factor (G-CSF): e.g., Filgrastim, to boost neutrophil count
• Antimicrobials: for prophylaxis during neutropenia
• Antifungals / antivirals: in high-risk cases

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🏥 Surgical Management

No primary surgical treatment, but surgery may be needed for:

• Abscess drainage: if leukopenia causes localized infection
• Splenectomy: rare, in cases of hypersplenism causing leukopenia
• Bone marrow/stem cell transplant: for severe, refractory bone marrow failure syndromes

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👩‍⚕️ Nursing Management

🔹 1. Infection Prevention

• Hand hygiene, strict aseptic technique
• Neutropenic precautions if WBC <1,000/mm³
• No fresh flowers, raw fruits/vegetables
• Limit visitors, use of masks

🔹 2. Monitoring

• Vitals every 4 hrs (especially temp >100.4°F)
• Skin/mucosal checks for signs of infection
• Monitor labs: WBC, ANC, cultures
• Watch for adverse drug reactions

🔹 3. Education

• Avoid crowded places, sick contacts
• Report fever or infection signs early
• Oral and personal hygiene
• Safe food practices

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⚠️ Complications

Complication	Description
Sepsis	From uncontrolled infection → shock, organ failure
Opportunistic infections	Pneumocystis pneumonia, fungal sepsis
Treatment interruptions	Chemo delay → affects cancer treatment outcomes
Emotional distress	Isolation and anxiety due to infection risk

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📌 Key Points to Remember

✅ Leukopenia = WBC count <4,000/mm³
✅ Neutropenia is the most clinically significant type
✅ Major danger = infections, which may have no early symptoms
✅ G-CSF (Filgrastim) is used to stimulate WBC production
✅ Always apply neutropenic precautions
✅ Patient education is crucial to prevent infection-related mortality
✅ Early recognition of fever can be life-saving

🌡️ Agranulocytosis

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📌 Definition:

Agranulocytosis is a severe and acute condition characterized by an extremely low level of granulocytes (especially neutrophils) in the blood, leading to increased susceptibility to infections.

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⚠️ Causes:

🔍 Cause Type	💡 Examples
💊 Drug-induced	Antithyroid drugs (e.g., methimazole), sulfonamides, clozapine, chemotherapy
🦠 Infection-related	Viral (EBV, hepatitis), bacterial sepsis
🧬 Autoimmune	Systemic lupus erythematosus
🧪 Toxic exposure	Benzene, radiation
👶 Congenital	Kostmann syndrome (rare genetic disorder)

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🧬 Types:

1. Primary Agranulocytosis – Genetic or idiopathic.
2. Secondary Agranulocytosis – Acquired due to drugs, infections, or toxins.

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🔁 Pathophysiology:

📉 Destruction or suppression of bone marrow stem cells ➡️
🚫 Decreased production of granulocytes (especially neutrophils) ➡️
🛡️ Reduced immune defense ➡️
🦠 High risk of opportunistic infections ➡️
💥 May lead to sepsis and multi-organ failure if untreated.

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🚨 Signs and Symptoms:

🩺 System	🧾 Symptoms
🌡️ General	High-grade fever, chills, fatigue
👄 Oral	Ulcers, sore throat, gingivitis
🦠 Infections	Respiratory, urinary, or systemic infections
❤️‍🩹 Skin	Non-healing wounds, rashes
🩸 Hematological	Low WBC count (<500 cells/µL)

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🧪 Diagnosis:

✅ CBC (Complete Blood Count) – Absolute neutrophil count < 500/µL
✅ Bone marrow biopsy – Hypocellular marrow
✅ Peripheral smear – Absence of granulocytes
✅ Blood cultures – Identify infections
✅ LFT, RFT – Monitor organ function during treatment

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💊 Medical Management:

🔹 Stop causative drug immediately
🔹 Broad-spectrum antibiotics – Prevent or treat infections
🔹 Granulocyte colony-stimulating factor (G-CSF) – Filgrastim, Pegfilgrastim
🔹 Antivirals or antifungals – If opportunistic infection present
🔹 IV fluids, nutritional support – To maintain hydration and nutrition

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🛠️ Surgical Management:

❌ Not usually required
✅ May be considered only if patient develops localized abscess or complications like infected necrotic tissue.

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🩺 Nursing Management:

🔹 Infection Control:

• Maintain strict hand hygiene 🧼
• Use PPE and isolate neutropenic patients 🛡️
• Avoid fresh flowers, raw fruits/veggies 🍓🚫

🔹 Monitoring:

• Check vital signs, especially temperature 🌡️
• Monitor CBC and neutrophil count 📊
• Observe for signs of infection 🔍

🔹 Patient Education:

• Avoid crowds, ill people 👥❌
• Report fever or sore throat immediately 📞
• Importance of medication adherence 💊

🔹 Supportive Care:

• Oral hygiene to prevent mouth ulcers 🪥
• Soft diet to avoid mucosal trauma 🍲
• Emotional support for anxiety or fear 🤝

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⚠️ Complications:

❗ Septicemia
❗ Multi-organ failure
❗ Recurrent infections
❗ Prolonged hospitalization
❗ Death if untreated

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🧷 Key Points:

✅ Agranulocytosis is a life-threatening condition due to lack of neutrophils
✅ Commonly caused by drugs, particularly chemotherapy and antipsychotics
✅ Prompt discontinuation of the causative agent is crucial
✅ G-CSF therapy plays a vital role in recovery
✅ Nursing care and infection prevention are central to management

🧬 Lymphomas

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📌 Definition:

Lymphoma is a type of blood cancer that originates in the lymphatic system, particularly affecting lymphocytes (a type of white blood cell). It leads to uncontrolled proliferation of abnormal lymphocytes, causing immune dysfunction and systemic spread.

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⚠️ Causes and Risk Factors:

🔍 Factor	💡 Details
🧬 Genetic mutations	Chromosomal translocations, inherited mutations
🦠 Infections	Epstein-Barr virus (EBV), HIV, HTLV-1, H. pylori (MALT lymphoma)
☢️ Radiation exposure	Prior cancer therapy, environmental
💊 Immunosuppressants	Organ transplant patients
🛡️ Autoimmune diseases	Rheumatoid arthritis, Sjögren’s syndrome
👪 Family history	Hereditary predisposition in some cases

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🧫 Types of Lymphoma:

1. 🔴 Hodgkin’s Lymphoma (HL)
   • Characterized by Reed–Sternberg cells
   • More localized spread, predictable
2. 🟠 Non-Hodgkin’s Lymphoma (NHL)
   • More common and diverse
   • Unpredictable spread, multiple subtypes (B-cell or T-cell)

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🔁 Pathophysiology:

1. Genetic mutation in lymphocyte precursor ➡️
2. Uncontrolled cell proliferation of abnormal lymphocytes ➡️
3. Accumulation in lymph nodes and organs ➡️
4. Suppression of normal immune function ➡️
5. Metastasis to liver, spleen, bone marrow, CNS in advanced stages

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🚨 Signs and Symptoms:

🩺 System	🧾 Symptoms
💢 Lymphatic	Painless swelling of lymph nodes (neck, armpit, groin)
🌡️ General	Unexplained fever, night sweats, weight loss (B symptoms)
🩸 Hematologic	Anemia, fatigue
🫁 Respiratory	Cough, chest pain, breathlessness (mediastinal mass)
🦴 Skeletal	Bone pain (with marrow involvement)
🤢 GI/Other	Nausea, abdominal pain (intestinal lymphoma), hepatosplenomegaly

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🧪 Diagnosis:

✅ Lymph node biopsy – Gold standard (to differentiate HL/NHL)
✅ Immunophenotyping – For lymphoma subtype classification
✅ CBC & ESR – Anemia, leukocytosis, elevated ESR
✅ Bone marrow aspiration/biopsy – Involvement check
✅ CT, PET scan – Staging and metastasis
✅ LDH levels – Marker for disease activity

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💊 Medical Management:

🔹 Chemotherapy:

• ABVD regimen (HL): Adriamycin, Bleomycin, Vinblastine, Dacarbazine
• CHOP regimen (NHL): Cyclophosphamide, Doxorubicin, Vincristine, Prednisone

🔹 Targeted therapy:

• Rituximab (Anti-CD20 monoclonal antibody for B-cell NHL)

🔹 Immunotherapy:

• Immune checkpoint inhibitors (e.g., nivolumab)

🔹 Radiotherapy:

• For localized tumors or residual mass

🔹 Stem cell transplantation:

• For relapsed or aggressive cases

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🛠️ Surgical Management:

❌ Not the mainstay of treatment
✅ May include:

• Diagnostic lymph node biopsy
• Splenectomy in some NHL cases (e.g., hypersplenism or splenic involvement)

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🩺 Nursing Management:

🔹 Monitoring and Assessment:

• Monitor vitals, CBC, and signs of infection or bleeding
• Assess lymph node swelling and systemic symptoms

🔹 Chemotherapy Care:

• Administer chemo with caution
• Monitor for side effects: nausea, mucositis, neutropenia
• Encourage hydration and nutrition

🔹 Infection Prevention:

• Neutropenic precautions
• Maintain hygiene and asepsis
• Avoid raw or uncooked foods

🔹 Psychosocial Support:

• Educate about disease, therapy, and prognosis
• Address anxiety, body image issues, and fear
• Encourage support group participation

🔹 Post-transplant care (if applicable):

• Monitor for graft-vs-host disease, infection, and rejection

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⚠️ Complications:

❗ Secondary infections (due to immunosuppression)
❗ Bone marrow suppression
❗ Organ failure (liver, kidney)
❗ Secondary malignancies (from chemo/radiation)
❗ Relapse or treatment resistance

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🧷 Key Points:

✅ Lymphomas are malignancies of lymphoid tissue
✅ Hodgkin’s Lymphoma is characterized by Reed-Sternberg cells
✅ B symptoms (fever, night sweats, weight loss) signal advanced disease
✅ Treatment includes chemotherapy, immunotherapy, radiation, and stem cell transplant
✅ Nursing care focuses on infection prevention, chemotherapy monitoring, and psychosocial support

🧬 Myelomas (Multiple Myeloma)

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📌 Definition:

Multiple Myeloma is a malignant proliferation of plasma cells in the bone marrow. These abnormal cells produce excess monoclonal immunoglobulin (M protein) and damage bones, kidneys, and the immune system.

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⚠️ Causes and Risk Factors:

🔍 Factor	💡 Details
🧬 Genetic mutations	Chromosomal abnormalities (e.g., 13q deletion, 17p deletion)
🌡️ Age	Common in individuals >60 years
☢️ Radiation/chemical exposure	Benzene, pesticides, Agent Orange
🧪 Chronic inflammation	Autoimmune diseases, infections
👪 Family history	First-degree relatives at higher risk
🦠 Viral infections	HIV, HHV-8

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🧫 Types of Myeloma:

1. 🔴 Multiple Myeloma – Most common form; affects multiple bones
2. 🟢 Solitary Plasmacytoma – Single tumor of plasma cells in bone/soft tissue
3. 🔵 Smoldering Myeloma – Asymptomatic but high risk of progression

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🔁 Pathophysiology:

1. Malignant plasma cells multiply in bone marrow ➡️
2. Produce abnormal monoclonal immunoglobulins (M-protein) ➡️
3. Bone destruction (via osteoclast activation) ➡️
4. Suppressed normal immunity & blood cell production ➡️
5. Renal damage due to light chain deposition ➡️
6. Hypercalcemia, anemia, fractures, infections

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🚨 Signs and Symptoms:

🎯 Remember the acronym: “CRAB”

Symbol	Symptom	Explanation
🦴 C	Calcium elevated (Hypercalcemia) – Nausea, confusion, constipation
🩸 R	Renal dysfunction – High creatinine, proteinuria
❤️ A	Anemia – Fatigue, pallor
🦴 B	Bone pain & lesions – Especially in spine, ribs; pathological fractures

Other signs:

• Recurrent infections 🦠
• Weight loss ⚖️
• Peripheral neuropathy ⚡

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🧪 Diagnosis:

✅ Serum protein electrophoresis (SPEP) – M spike (monoclonal protein)
✅ Urine protein electrophoresis (UPEP) – Bence-Jones proteins
✅ Serum free light chain assay
✅ Bone marrow biopsy – >10% clonal plasma cells
✅ X-rays / MRI / PET-CT – Lytic bone lesions
✅ CBC, Calcium, Renal function tests – For CRAB features

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💊 Medical Management:

🔹 Chemotherapy:

• Bortezomib, Lenalidomide, Cyclophosphamide
• Dexamethasone (steroid)

🔹 Targeted therapy & Immunotherapy:

• Monoclonal antibodies (e.g., Daratumumab)
• CAR-T cell therapy (in relapsed cases)

🔹 Bisphosphonates:

• Zoledronic acid to prevent bone fractures

🔹 Plasmapheresis:

• For hyperviscosity syndrome due to high protein levels

🔹 Stem Cell Transplantation:

• Autologous transplant for eligible patients

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🛠️ Surgical Management:

❌ Not primary treatment, but:

• May include vertebroplasty for spinal fractures
• Surgical stabilization of pathologic fractures
• Radiotherapy for local plasmacytomas

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🩺 Nursing Management:

🔹 Monitoring and Assessment:

• Assess for signs of bone pain, infection, renal dysfunction
• Monitor CBC, calcium, and kidney function

🔹 Infection Prevention:

• Neutropenic precautions
• Avoid exposure to infectious people

🔹 Supportive Care:

• Pain management 💊
• Encourage hydration for kidney protection 💧
• Fall precautions due to bone weakness ⚠️

🔹 Patient Education:

• Educate about symptom recognition
• Teach medication adherence and side effect management
• Importance of regular lab monitoring

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⚠️ Complications:

❗ Renal failure
❗ Bone fractures and spinal cord compression
❗ Hypercalcemia crisis
❗ Infections
❗ Anemia-related complications
❗ Secondary leukemia (due to treatment)

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🧷 Key Points:

✅ Multiple myeloma is a plasma cell cancer affecting bones, kidneys, and immune function
✅ CRAB criteria are essential in diagnosis
✅ Treatment includes chemo, immunotherapy, bisphosphonates, and stem cell transplant
✅ Early diagnosis and supportive care can improve quality of life
✅ Nursing care involves monitoring labs, infection prevention, hydration, and patient education.