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Hemolytic anemia

Hemolytic anemia. Rakesh Biswas MD, Professor, Department of Medicine, People's College of Medical Sciences, Bhanpur, Bhopal, India. Young man of 19 Complains of giddiness weakness, pallor Examination reveals a spleen mild lemon yellow sclera.

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Hemolytic anemia

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  1. Hemolytic anemia Rakesh Biswas MD, Professor, Department of Medicine, People's College of Medical Sciences, Bhanpur, Bhopal, India

  2. Young man of 19Complains of giddiness weakness, pallorExamination reveals a spleenmild lemon yellow sclera

  3. How shall you investigate to find out the cause of the problem?

  4. Laboratory investigations:Severe normochromic, normocytic anemia (hemoglobin level of 6.4 g/dLReticulocyte count of 12.2%. Blood film:

  5. Bilirubin level of 2.5 mg/dL, Lactate dehydrogenase (LDH) of 2140 IU/L,Haptoglobin below 7 mg/dL

  6. Introduction • Mean life span of a RBC-120days • Removed Extravascularly by- Macrophages of RE system

  7. Hemolytic Anemia • Definition: • Those anemias which result from an increase in RBC destruction • Classification: • Congenital / Hereditary • Acquired

  8. Hemoglobinuria

  9. Ref : Harrison’s

  10. Features of HEMOLYSIS Bilirubin LDH Reticulocytes, n-RBC Haptoglobulins +ve Urinary hemosiderin, Urobilinogen Blood Film Spherocytes No spherocytes Fragmentation DCT +ve DCT –ve AI Hemolysis H. Sherocytosis Malaria, Clostidium Hereditery enzymopathies Microangiopathic, Traumatic

  11. Red Cell Membrane Defects 1.Hereditary Spherocytosis • Usually inherited as AD disorder • Defect: Deficiency of Beta Spectrin or Ankyrin Loss of membrane in Spleen & RES becomes more spherical Destruction in Spleen

  12. RBC Membrane

  13. C/F: Asymptomatic Fluctuating hemolysis Splenomegaly Pigmented gall stones- 50%

  14. Complications • Clinical course may be complicated with Crisis: • Hemolytic Crisis: associated with infection • Aplastic crisis: associated with Parvovirus infection

  15. Inv: Test will confirm Hemolysis P Smear: Spherocytes Osmotic Fragility: Increased Screen Family members

  16. Osmotic Fragility

  17. Management: Folic Acid 5mg weekly, prophylaxis life long Spleenectomy Blood transfusion in Ac, severe hemolytic crisis

  18. 2.Hereditary Elliptocytosis • Equatorial Africa, SE Asia • AD / AR • Functional abnormality in one or more anchor proteins in RBC membrane- Alpha spectrin , Protein 4.1 • Usually asymptomatic • Mx: Similar to H. spherocytosis • Variant: 3.SE-Asian ovalocytosis: • Common in Malaysia , Indonesia… • Asymptomatic-usually • Cells oval , rigid ,resist invasion by malarial parasites

  19. Elliptocytosis

  20. Red Cell Enzymopathies • Physiology: • EM pathway: ATP production • HMP shunt pathway: NADPH & Glutathione production

  21. 1. Glucose-6-Phosphate Dehydrogenase( G6PD ) Deficiency Pivotal enzyme in HMP Shunt & produces NADPH to protect RBC against oxidative stress Most common enzymopathy -10% world’s population Protection against Malaria X-linked

  22. (Oxidisedform) (Reduced form)

  23. Clinical Features: Acute drug induced hemolysis: Aspirin, primaquine, quinine, chloroquine, dapsone…. Chronic compensated hemolysis Infection/acute illness Neonatal jaundice Favism

  24. Inv: e/o non-spherocytic intravascular hemolyis P. Smear: Bite cells, blister cells, irregular small cells, Heinz bodies, polychromasia G-6-PD level Treatment: Stop the precipitating drug or treat the infection Acute transfusions if required

  25. 2. PyruvateKinase Deficiency AR Deficient ATP production, Chronic hemolytic anemia Inv; P. Smear: Prickle cells Decreased enzyme activity Treatment: Transfusion may be required

  26. Hemolobinopathies…

  27. Autoimmune Hemolytic Anemia • Result from RBC destruction due to RBC autoantibodies: Ig G, M, E, A • Most commonly-idiopathic • Classification • Warm AI hemolysis:Ab binds at 37degree Celsius • Cold AI Hemolysis: Ab binds at 4 degree Celsius

  28. 1.Warm AI Hemolysis: Can occurs at all age groups F > M Causes: 50% Idiopathic Rest - secondary causes: 1.Lymphoid neoplasm: CLL, Lymphoma, Myeloma 2.Solid Tumors: Lung, Colon, Kidney, Ovary, Thymoma 3.CTD: SLE,RA 4.Drugs: Alpha methyl DOPA, Penicillin , Quinine, Chloroquine 5.Misc: UC, HIV

  29. MACROCYTE SPHEROCYTE IMMUNOHEMOLYTIC ANEMIA

  30. complement Direct antiglobulin testdemonstrating the presence of autoantibodies (shown here) or complement on the surface of the red blood cell.

  31. Inv: e/o hemolysis, MCV P Smear: Microspherocytosis, n-RBC Confirmation: Coomb’s Test / Antiglobulin test Treatment Correct the underlying cause Prednisolone 1mg/kg po until Hb reaches 10mg/dl then taper slowly and stop Transfusion: for life threatening problems If no response to steroids  Spleenectomy or, Immunosuppressive: Azathioprine, Cyclophosphamide

  32. 2. Cold AI Hemolysis Usually Ig M Acute or Chronic form Chronic: C/F: Elderly patients Cold , painful & often blue fingers, toes, ears, or nose ( Acrocyanosis) Inv: e/o hemolysis P Smear: Microspherocytosis Ig M with specificity to I or I Ag

  33. Other causes of Cold Agglutination: Infection: Mycoplasma pneumonia, Infec Mononucleosis PCH : Rare cause seen in children in association with cong syphilis

  34. Treatment: Treatment of the underlying cause Keep extremities warm Steroids treatment Blood transfusion

  35. Non-Immune Acquired Hemolytic Anemia 1. Mechanical Trauma A). Mechanical heart valves, Arterial grafts: cause shear stress damage B).March hemoglobinuria: Red cell damage in capillaries of feet C). Thermal injury: burns D). Microangiopathic hemolytic anemia (MAHA): by passage of RBC through fibrin strands deposited in small vessels  disruption of RBC eg: DIC,PIH, Malignant HTN,TTP,HUS

  36. TRAUMATIC HEMOLYSIS

  37. Acquired hemolysis 2.Infection F. malaria: intravascular hemolysis: severe called ‘Blackwater fever’ Cl. perfringens septicemia 3.Chemical/Drugs: oxidant denaturation of hemoglobin Eg: Dapsone, sulphasalazine, Arsenic gas, Cu, Nitrates & Nitrobenzene

  38. The direct antiglobulin test was positive for complement (C3d) (++), and IgG (++-). Also was positive for agglutinins of IgM type and had a titer of 1:1024.

  39. Serologies for human immunodeficiency virus, hepatitis B and C viruses, and Mycoplasma pneumoniae were negative. Rheumatoid factor and antinuclear antibodies were undetectable.

  40. Prednisone therapy was started at a dose of 1 mg/kg intravenously, daily. Hemoglobin level rose to 11 g/dL, concomitantly with the improvement of hemolytic signs.

  41. A reduction of positivity of both direct and indirect antiglobulin tests (polyvalent serum + ; C3d + ; IgG+ ), as well as a reduction of cold agglutinin titers (1:128), was observed 8 weeks after corticosteroid therapy.

  42. Three months later, corticosteroids were tapered to a maintenance dose of 25 mg daily. Hemolysis recurred again with the fall of hemoglobin to 7 g/dL.

  43. The direct antiglobulin test recurred positive for polyvalent serum (+++), complement (+++), and IgG (+++), while cold agglutinin titers again became strongly positive (1:256).

  44. Immunophenotyping of bone marrow cells showed that 10% of all the cells were CD20 and CD19 positive.

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