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Warm Autoimmune Hemolytic Anemia

Warm Autoimmune Hemolytic Anemia . Lisa Rose-Jones, MD Monday, Aug 24th. Autoimmune Hemolytic Anemia: Warm Agglutinins. Due to IgG antibodies that react with protein antigens on RBC surface @ body temperature

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Warm Autoimmune Hemolytic Anemia

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  1. Warm Autoimmune Hemolytic Anemia Lisa Rose-Jones, MD Monday, Aug 24th

  2. Autoimmune Hemolytic Anemia: Warm Agglutinins • Due to IgG antibodies that react with protein antigens on RBC surface @ body temperature • Extravascular (red blood cells are destroyed in the spleen and other reticuloendothelial organs) hemolysis

  3. HemolysisDiagnosis: • Typically find Hgb in range of 7-10 g/dL (Hct 21-30%) • Hemolysis: Increased Reticulocyte*/LDH/Indirect Bilirubin; Decreased Haptoglobin *Some w/ acute hemolysis may have reticulocytopenia from lag in marrow responsiveness 2/2 hemolytic stress

  4. Warm Hemolysis Dxcontd… • Peripheral smear show spherocytosis (appear abnormally small and will lack the central pallor)

  5. Direct Coombs’ test • Test for dignosis of warm AIHA • Adherent proteins are washed free from pt’s RBCs, mixed w/ antiserum or monocolonal Abs against different Immunoglobulins (IgG, C3d)= & if present on RBC surface degree agglutination quantitated by ELISA

  6. Differential Diagnosis ~ Drug Induced: Cephalosporins, PCN & derivatives, NSAIDs, Quinidine ~Cold Agglutinins, (cold reacting IgM antibodies). Acral cynaosis

  7. Pathogenesis of RBC destruction in AIHA: Immunoadherence: RBC (target cell) affixed to destructive cell (phagocyte) through immunoprotein molecule on each cell ~ingestion may be partial, resulting in RBC w/ reduced surface:vol ratio = spherocyte, b/c reduced deformability many can’t cross splenic sinuses slits=destroyed

  8. Why Autoantibody Production? • B cell clones appear to be altered to produce these antibodies at high pathogenic titers • Exacerbated by immune reaction of microbial infection?, viral infections (HIV, Mono) • Rxn to blood transfusion or transplant my initiate this process

  9. Why?....... • Higher incidence of autoantibodies associated w/ Systemic Lupus • 11% of patients’ w/ Chronic Lymphocytic Leukemia (CLL), likely b/c of again abnormal B cells (neoplastic proliferation)

  10. Treatment Goals: ** Reduce the amount of Antibody being produced & reduce its effiency in destroying RBCs ~Success ≠ Cure, typically still evidence of persistent activity of underlying process. Control degree of anemia

  11. Reduce Antibody Response • Corticosteroids (1mg/kg Prednisone), see response in 1-3 wks • Cytotoxic Agents: Azathioprine & Cyclophosphamide, given if lack response/inability tolerate #1. Response seen ~ 1 month. Case reports in resistant cases use of Cyclosporine, Mycophenolate, and anti-CD20 antibodies (Rituximab)

  12. Reduce Antibody Effectiveness • Splenectomy(removing primary site of destruction) • IVIG: only occ effective (40%) in tx of AIHA refractory to conventional therapy w/ Prednisone & splenectomy (reducing the interaction b/w spleen macrophages & Ab coated RBCs)

  13. RBC Transfusions: • Tested for alloantibodies* (develops following pregnancy or prior transfusions) & autoantibodies • Experience has indicated most pts will tolerate serologically incomplatible (atuoantibodies) blood

  14. THE END!!

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