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Haemolytic anaemia by Afa

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  • 1. Haemolytic anaemias Atifa Ambreen
  • 2. Introduction • Mean life span of a RBC-120days • Removed Extravascularly by- Macrophages of RE system.
  • 3. Intravascular haemolysis occurs in some pathological disorders
  • 4. Normal red blood cell breakdown.This takes place extravascularly in the macrophagesof the reticuloendothelilial system
  • 5. Hemolytic Anemia • Definition: – Those anemias which result from an increase in RBC destruction • Classification: – Congenital / Hereditary – Acquired
  • 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. 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. 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. 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. Insert Title Text Here • Extravascular Intravascular • URINE • Bilirubin 0 0 • Hemosiderin 0 + • Hemoglobin 0 + in severe cases
  • 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
  • 12. Insert Title Text Here
  • 13. Clinical features • Asymptomatic • Fluctuating hemolysis • Splenomegaly • Pigmented gall stones- 50%
  • 14. Clinical course may be complicated with Crisis: – Hemolytic Crisis: associated with infection – :Aplastic crisis associated with Parvovirus infection
  • 15. Investigations – Test will confirm Hemolysis – Periphral Smear: Spherocytes – Osmotic Fragility: Increased Screen Family members
  • 16. Insert Title Text Here
  • 17. • 2.Hereditary Elliptocytosis Functional abnormality in one or more anchor proteins in RBC membrane- Alpha or beta spectrin , Protein 4.1 • Usually asymptomatic
  • 18. Insert Title Text Here
  • 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. Membrane abnormalities - Enzymopathies • Deficiencies in Hexose Monophosphate Shunt – Glucose 6-Phosphate Dehydrogenase Deficiency • Deficiencies in the EM Pathway – Pyruvate Kinase Deficiency
  • 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
  • 22. Insert Title Text Here
  • 23. Investigations –P. Smear: Bite cells, blister cells, irregular small cells, Heinz bodies, polychromasia –G-6-PD level
  • 24. 2. Pyruvate Kinase Deficiency –Deficient ATP production, Chronic hemolytic anemia –Investigations • Priphral Smear: Prickle cells • Decreased enzyme activity
  • 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. 1.Warm Autoimmune hymolysis • Usually IgG Idiopathic Secondary causes SLE,CLL,lymphomas,Drugs(e.g.Methyldopa)
  • 27. Insert Title Text Here MACROCYTE SPHEROCYTE IMMUNOHEMOLYTIC ANEMIA
  • 28. Investigation – e/o hemolysis, MCV – P Smear: Microspherocytosis, n-RBC – Confirmation: Coomb’s Test / Antiglobulin test
  • 29. Direct Antiglobulin test demonstrating the presence of autoantibodies or complement on the surface of the red blood cell.
  • 30. Cold Autoimmune haemolysis • Usually IgM Infections Mycoplasm pneumonia,infectiousmononucleosis lymphoma proxysmal cold haemoglobinuria
  • 31. Investigation • Periphral Smear: Microspherocytosis Ig M with specificity to I or I Ag
  • 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. THANK YEW ... !!!

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