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Warm Autoimmune Hemolytic Anemia

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Warm Autoimmune Hemolytic Anemia Lisa Rose-Jones, MD Monday, Aug 24th Autoimmune Hemolytic Anemia: Warm Agglutinins Due to IgG antibodies that react with protein ... – PowerPoint PPT presentation

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Title: Warm Autoimmune Hemolytic Anemia


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 _at_ body temperature
  • Extravascular (red blood cells are destroyed in
    the spleen and other reticuloendothelial organs)
    hemolysis

3
Hemolysis Diagnosis
  • 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 Dx contd
  • 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 pts 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 surfacevol ratio spherocyte, b/c
    reduced deformability many cant cross splenic
    sinuses slitsdestroyed

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
  1. Corticosteroids (1mg/kg Prednisone), see response
    in 1-3 wks
  2. 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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