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HEMOLYTIC ANEMIA

HEMOLYTIC ANEMIA. DR. Subhan Ali R. HEMOLYTIC ANEMIA. DEFINITION CAUSES APPROACH TO DIAGNOSIS DIAGNOSIS. DEFINITION. Anemia due to inability of the bone marrow to compensate for the degree of destruction of RBC. Normal RBC destruction. Life span 100- 120 days

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HEMOLYTIC ANEMIA

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  1. HEMOLYTIC ANEMIA DR. Subhan Ali R

  2. HEMOLYTIC ANEMIA • DEFINITION • CAUSES • APPROACH TO DIAGNOSIS • DIAGNOSIS

  3. DEFINITION • Anemia due to inability of the bone marrow to compensate for the degree of destruction of RBC

  4. Normal RBC destruction • Life span 100- 120 days • Towards the end red cell surface changes make them susceptible to phagocytosis by RES • Energy derived from glucose breakdown maintains red cell integrity

  5. Hb metabolism • Phagocytosed RBCS release Hb • Broken down within RES • Globin back to metabolic protein pool • Porphyrin ring of heam cleaved by HO • Yields biliverdin and CO • Reduced to bilirubin

  6. Haem degradation

  7. Causes of hemolysis • Extra vascular • Intra vascular

  8. Extra vascular • 80-90% in Spleen • Others sites are bone marrow reticuloendothelial cells • Characteristics • (1) ↑ Bilirubin - blood • (2) ↑ Stercobilinogen – stool • (3) ↑ Urobilinogen – urine • (4) ↑ Iron store – bone marrow

  9. Intra vascular • RBC destroyed while in circulation • Minor pathway • Characteristics • Haemoglobinemia • Haemoglobinuria • Haemosiderinuria • Serum haptoglobin

  10. Causes • Two types • (A) Hereditary • (B) Acquired

  11. Hereditary • (A) Defect in RBC membrane • (B) Defect in globins synthesis • (C) Enzyme deficiency of glycolytic pathway • (D) Enzyme deficiency of pentose phosphate pathway • (E) Enzyme deficiency of RBC nucleotide metabolism

  12. Acquired • (A) Immunohemolytic anemia • (B) Fragmentation syndrome • (C) Paroxysmal nocturnal haemoglobinuria • (D) Drugs & chemicals • (E) Thermal injury • (F) Infections

  13. Defect in RBC membrane • Hereditary spherocytosis • Hereditary elliptocytosis • Stomatocytosis • Acanthocytosis (Abetalipoproteinemia)

  14. Defect in globins synthesis • Beta Thalassemias • Alpha Thalassemias • Sickling syndromes • Unstable Hb disease

  15. Enzyme deficiency of glycolytic pathway • Pyruvate kinase deficiency • Hexokinase deficiency

  16. Enzyme deficiency of pentose phosphate pathway • Glucose-6-Phosphate Dehydration deficiency (G-6-PD)

  17. Enzyme deficiency of RBC nucleotide metabolism • Pyrimidine-5’-nucleotidase deficiency

  18. Immunohemolytic anemia • Autoimmune haemolytic anaemias • Due to warm antibodies • Idiopathic • Secondary • Due to cold antibodies • Cold agglutinin disease • Paroxysmal cold haemoglobinuria • Haemolytic disease of newborn

  19. Fragmentation syndrome • Haemolytic uremic syndrome • Thrombotic thrombocytopenic purpura • Disseminated intavascular coagulation • Prosthetic cardiac valves

  20. Drugs Oxidant drugs Primaquine Dapson Chemicals Naphthalene Nitrites and Nitrates Oxidizing chemicals Drugs & chemicals

  21. Thermal injury • Burn • Electric shock • Heat stroke

  22. Infections • Clostridium welchi septicemia • Bartonellosis • Cholera

  23. APPROACH TO DIAGNOSIS • History • Clinical Examination • Laboratory Evaluation

  24. History • Complaints • Age at onset • Development of pallor • Duration of disease • Jaundice • Exposers to chemicals or drugs • Infection , fever • Color of urine • Color of stool • Family history

  25. Clinical Examination • Pallor • Jaundice • Gall stone • Spleenomegaly • Skeletal abnormalities • Leg ulcers

  26. Laboratory Evaluation • Peripheral blood examination • Bone marrow examination • Biochemical finding • Specific test

  27. Peripheral blood examination • RBC :- POLYCHROMATOPHILIA NUCLEATED RBC RETICULOCYTOSIS POIKILOCYTOSIS SPHEROCYTE, SICKEL CELL, TARGET CELL, SCHISTOCYTES, ACANTHOCYTE • WBC :- NEUTROPHILIA • PLATELETS :- ↑ GIANT PLATELETS

  28. Bone marrow examination • ERYTHROPOISIS – HYPERCELLULAR REVERSE M:E RATIO – 1 : 1 to1 : 6 (NORMAL 3-7 : 1) NORMOBLASTIC REACTION PREPONDERANCE OF NORMOBLAST DYSERYTHROPOISIS (A) HOWEL JOLLY BODIES (B) ABNORMAL HEMOGLOBNISATION (C) IRREGULAR NUCLEAR BORDER (D) ABNORMAL MITOSIS • MYELOPOISIS – NORMAL • MEGAKARYOPOISIS – NORMAL

  29. Biochemical finding • S. BILIRUBIN :- ↑ • URINE UROBILINOGEN :- ↑ • RATE OF BILIRUBIN PRODUCTION :- ↑ • S. LDH LEVELS :- ↑ • S. HAPTOGLOBIN :- ↓ • LIFE SPAN OF RBC :- ↓

  30. Specific test • Hemoglobinopathies • Fetal Hb estimation • Hb electrophoresis • Sickling test • Reticulocyte preparation • Cytochemical stain for HbF • Heinz body preparation

  31. Specific test • Spherocytosis • osmotic fragility • Acidified glycerol lysis test • Immune hemolytic • coomb test • Direct & indirect

  32. Specific test • G-6-PD deficiency • Methaemoglobin reduction test • Fluorescent spot test • Quantitative assay • Heinz body test • Ascorbate - cyanide test

  33. Specific test • P.K. deficiency • Autohemolysis test • Paroxysmal nocturnal haemoglobinuria • Ham’s acidified serum test • CD 59, CD 55

  34. Specific test • Bite cell haemolytic anaemias :- • Heinz body test • Bite cell in PS • Methaemoglobinaemia • Spectroscopy • Paroxysmal cold haemoglobinuria • Landsteiner;s test

  35. THANK YOU

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