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Key Features

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Essentials of Diagnosis
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  • Pallor, fatigue, jaundice, and dark urine

  • Splenomegaly

  • Positive direct antiglobulin test

  • Reticulocytosis and spherocytosis

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General Considerations
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  • One of the more common causes of acute anemia after the first year but rare during first 4 months of life

  • May arise as a primary disorder

  • May complicate

    • An infection (hepatitis, upper respiratory tract infections, Epstein-Barr [EBV] mononucleosis, or cytomegalovirus [CMV] infection)

    • Systemic lupus erythematosus and other autoimmune syndromes

    • Immunodeficiency states, including autoimmune lymphoproliferative syndrome (ALPS)

    • Malignancies, albeit very rarely

  • Drugs may induce antibody-associated hemolytic anemia

  • Third-generation cephalosporins have become a common cause

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Clinical Findings

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  • Onset is usually sudden manifested by weakness, pallor, dark urine, and fatigue

  • However, in some cases, onset can be more gradual and insidious

  • Jaundice is a prominent finding

  • Splenomegaly is often present

  • Clinical evidence of an underlying disease may be present

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Diagnosis

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  • Anemia is normochromic and normocytic and may vary from mild to severe (hemoglobin concentration < 5 g/dL)

  • Reticulocyte count and index are usually increased but occasionally are normal or low

  • Spherocytes and nucleated red cells may be seen on the peripheral blood smear

  • Although leukocytosis and elevated platelet counts are common, thrombocytopenia occasionally occurs

  • Intravascular hemolysis is indicated by hemoglobinemia or hemoglobinuria

  • Examination of bone marrow shows marked erythroid hyperplasia and hemophagocytosis, but is seldom required for the diagnosis

  • In almost all cases, the direct and indirect antiglobulin tests are positive

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Treatment

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  • Warm autoimmune hemolytic anemia (AIHA) (in which hemolysis is mostly extravascular)

    • Most patients (50–80%) respond to prednisone (2 mg/kg/d)

    • Patients may respond to intravenous immune globulin (IVIg) 1 g/kg/d for 2 days

    • Rituximab

      • May be a successful alternative in severe cases

      • However, should be avoided when anemia is associated with ALPS

    • Splenectomy

      • Remission with splenectomy may be as high as 50–60%

      • However, should be considered only for patients older than 5 years who are refractory or resistant to first-line therapies

    • In cases unresponsive to more conventional therapy, following immunosuppressive agents may be tried alone or in combination with corticosteroids

      • Mycophenolate

      • Sirolimus

      • Cyclosporine

      • Cyclophosphamide

      • Azathioprine

      • Busulfan.

    • Transplantation, especially when hemolysis is secondary, has been used successfully in small numbers of cases

  • Cold AIHA and paroxysmal cold hemoglobinuria

    • Less likely to respond to corticosteroids or IVIg

    • Supportive care alone may be sufficient since these syndromes are most apt to be associated with infections and have an acute, self-limited course

    • Plasma exchange may be effective in severe cold (IgM) AIHA because the offending antibody has an intravascular distribution

    • Transfusion

      • May be necessary because of the complications of severe anemia

      • Should be used only when there is no alternative

    • Heparin should be considered in patients with severe intravascular hemolysis who may have associated disseminated intravascular coagulation

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Outcome

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Complications
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  • Anemia may be very severe and result in ...

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