Haemolytic anaemia by Afa

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Haemolytic anaemia by Afa

  1. 1. Haemolytic anaemias Atifa Ambreen
  2. 2. Introduction • Mean life span of a RBC-120days • Removed Extravascularly by- Macrophages of RE system.
  3. 3. Intravascular haemolysis occurs in some pathological disorders
  4. 4. Normal red blood cell breakdown.This takes place extravascularly in the macrophagesof the reticuloendothelilial system
  5. 5. Hemolytic Anemia • Definition: – Those anemias which result from an increase in RBC destruction • Classification: – Congenital / Hereditary – Acquired
  6. 6. Classification of Hemolytic Anemias • Hereditary Memeberane Hereditary spherocytosis,Hereditary elliptocytosis Metabolism G6PD Deficiency,pyruvate kinase deficiency Haemoglobin Genetic abnormalities(Hb S,Hb C,unstable)
  7. 7. Acquired • Immune Autoimmune warm antibody type cold antibody type Alloimmune haemolytic transfusion reaction haemolytic disease of newborn Allografts,especially stem cell transplantation Drug associated
  8. 8. Insert Title Text Here • Red cell fragmentation syndromes March haemoglobinuria infections Malaria,Clostridia Chemical and physical agents Especially drugs,burns Secondary liver and renal disease proxysmal nocturnal haemoglobinuria(PNH)
  9. 9. Laboratory evaluation of Haemolysis • HEMATOLOGIC Extravascular Intravascular Routine blood film Polychromatophilia Polychromatophilia • Reticulocyte count Increase increase • Bone marrow examination Erythroid Erythroid hyperplasia hyperplasia • PLASMA OR SERUM • Bilirubin unconjugated unconjugated • Haptoglobin Dec,Absent Absent • Plasma hemoglobin N/inc. Increase • Lactate dehydrogenase Variable increase
  10. 10. Insert Title Text Here • Extravascular Intravascular • URINE • Bilirubin 0 0 • Hemosiderin 0 + • Hemoglobin 0 + in severe cases
  11. 11. Red Cell Membrane Defects 1.Hereditary Spherocytosis – Usually inherited as Autosomal Dominent disorder – Defect: Deficiency of Beta Spectrin or Ankyrin  Loss of membrane in Spleen & RES becomes more spherical Destruction in Spleen
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  13. 13. Clinical features • Asymptomatic • Fluctuating hemolysis • Splenomegaly • Pigmented gall stones- 50%
  14. 14. Clinical course may be complicated with Crisis: – Hemolytic Crisis: associated with infection – :Aplastic crisis associated with Parvovirus infection
  15. 15. Investigations – Test will confirm Hemolysis – Periphral Smear: Spherocytes – Osmotic Fragility: Increased Screen Family members
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  17. 17. • 2.Hereditary Elliptocytosis Functional abnormality in one or more anchor proteins in RBC membrane- Alpha or beta spectrin , Protein 4.1 • Usually asymptomatic
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  19. 19. South-East Asian ovalocytosis: • Caused by a nine amino acid deletion at he junction of the cytoplasmic and transmemberane domains of the band 3 protein • Asymptomatic-usually • Cells oval , rigid ,resist invasion by malarial parasites • Common in malaysia , indonesia
  20. 20. Membrane abnormalities - Enzymopathies • Deficiencies in Hexose Monophosphate Shunt – Glucose 6-Phosphate Dehydrogenase Deficiency • Deficiencies in the EM Pathway – Pyruvate Kinase Deficiency
  21. 21. Glucose 6-Phosphate Dehydrogenase • Regenerates NADPH, allowing regeneration of glutathione • Protects against oxidative stress • Lack of G6PD leads to hemolysis during oxidative stress – Infection – Medications – Fava beans • Oxidative stress leads to Heinz body formation,  extravascular hemolysis
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  23. 23. Investigations –P. Smear: Bite cells, blister cells, irregular small cells, Heinz bodies, polychromasia –G-6-PD level
  24. 24. 2. Pyruvate Kinase Deficiency –Deficient ATP production, Chronic hemolytic anemia –Investigations • Priphral Smear: Prickle cells • Decreased enzyme activity
  25. 25. Autoimmune Hemolytic Anemia • Result from RBC destruction due to RBC autoantibodies: Ig G, M, E, A • Most commonly-idiopathic • Classification – Warm Autoimmune haemolysis:Ab binds at 37degree Celsius – Cold Autoimmune haemolysis: Ab binds at 4 degree Celsius
  26. 26. 1.Warm Autoimmune hymolysis • Usually IgG Idiopathic Secondary causes SLE,CLL,lymphomas,Drugs(e.g.Methyldopa)
  27. 27. Insert Title Text Here MACROCYTE SPHEROCYTE IMMUNOHEMOLYTIC ANEMIA
  28. 28. Investigation – e/o hemolysis, MCV – P Smear: Microspherocytosis, n-RBC – Confirmation: Coomb’s Test / Antiglobulin test
  29. 29. Direct Antiglobulin test demonstrating the presence of autoantibodies or complement on the surface of the red blood cell.
  30. 30. Cold Autoimmune haemolysis • Usually IgM Infections Mycoplasm pneumonia,infectiousmononucleosis lymphoma proxysmal cold haemoglobinuria
  31. 31. Investigation • Periphral Smear: Microspherocytosis Ig M with specificity to I or I Ag
  32. 32. Alloimmune • Induced by red cell antigens Haemolytic transfusion reactions haemolytic disease of the new born post stem cell grafts Drug induced Drug-red cell membrane complex immune complex
  33. 33. THANK YEW ... !!!

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