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  • • Fatigue, pallor, jaundice

    • Splenomegaly

    • Persistent anemia and reticulocytosis

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Epidemiology

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  • • Autoimmune disorder that is either idiopathic (40-50%) or secondary to drug exposure, connective tissue diseases, or lymphoproliferative disorders

    • Classified according to the optimal temperature at which autoantibodies react with the red cell surface (warm or cold antibodies)

    • -Warm antibody: IgG autoantibodies directed against the Rh locus on the erythrocyte coat the cell and bind to IgG specific Fc receptors on macrophages in the spleen

      -Cold antibody: IgM autoantibodies are directed against the I red cell antigen; hemolysis occurs intravascularly and not within the spleen

    • Most common after age 50; occurs twice as often in women than in men

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Symptoms and Signs

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  • • Fatigue

    • Mild jaundice

    • Fever

    • Splenomegaly

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Laboratory Findings

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  • • Acute onset normocytic normochromic anemia

    • Reticulocytosis (> 10%)

    • Erythroid hyperplasia of the marrow

    • Elevation of serum indirect bilirubin

    • Serum haptoglobin is usually low or absent

    • Positive direct Coombs test

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  • • Rarely, a sudden, severe onset hemolytic anemia produces hemoglobinuria, renal tubular necrosis, and a 40-50% death rate

    • May be associated with systemic lupus erythematosus and chronic lymphocytic leukemia

    • Drugs commonly implicated include methyldopa, penicillin, quinidine

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Rule Out

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  • • Other causes of hemolytic anemia

    • -Hereditary hemolytic anemias

      -Thrombotic thrombocytopenic purpura

      -Disseminated intravascular coagulation

      -Infection

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  • • CBC count

    • Serum bilirubin

    • Reticulocyte count

    • Peripheral blood smear

    • Serum haptoglobin

    • Bone marrow biopsy

    • Coombs test

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When to Admit

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  • • Severe anemia

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When to Refer

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  • • All cases should be managed in conjunction with a hematologist

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  • • Drug-induced hemolytic anemia: Terminate exposure to the agent.

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Surgery

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  • • Splenectomy is indicated for patients with warm-antibody hemolysis meeting the following criteria:

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Indications

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  • • Failure to respond to 4-6 weeks of high-dose corticosteroid therapy

    • Relapse when corticosteroids are withdrawn

    • Contraindications to corticosteroid therapy

    • Chronic high-dose corticosteroid therapy

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Contraindications

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  • • Cold-antibody hemolytic anemia

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Medications

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  • • Corticosteroids

    • IV immune globulin

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Treatment Monitoring

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  • • CBC count

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Complications

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  • • Pigment gallstones

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Prognosis

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  • • 75% remission rate with medical treatment; 25% are permanent

    • Relapses may occur after splenectomy

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References

Beutler E et al. Hemolytic anemia. Semin Hematol. 1999;36:38.  [PubMed: 10595753]

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