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Key Features

  • Acquired disease is rare; in adults, it is usually idiopathic

  • Cases have been seen in association with systemic lupus erythematosus, chronic lymphocytic leukemia (CLL), lymphomas, or thymoma

  • Some drugs (phenytoin, chloramphenicol) may cause red cell aplasia

  • Rarely, anti-erythropoietin antibodies cause pure red cell aplasia in patients who are treated with recombinant erythropoietin

Clinical Findings

  • The only signs are those of anemia unless the patient has an associated autoimmune or lymphoproliferative disorder

  • Transient episodes of red cell aplasia are probably common in response to viral infections, especially parvovirus infections

  • Acute episodes go unrecognized unless the patient has a chronic hemolytic disorder or a chronic immunocompromised state, in which case the hematocrit may fall precipitously

Diagnosis

  • Anemia is often severe and normochromic, with low or absent reticulocytes

  • Red blood cell morphology is normal, and the myeloid and platelet lines are unaffected

  • Bone marrow

    • Normocellular with markedly reduced or absent erythroid progenitors

    • All non-erythroid elements are present and normal

  • The bone marrow karyotype is normal on standard banding cytogenetics

  • Chest imaging may reveal a thymoma

Treatment

  • Possible offending drugs should be stopped

  • Most patients will require red blood cell transfusion support

  • Resection of thymoma may result in amelioration of anemia

  • High-dose intravenous immune globulin has produced excellent responses in a small number of cases, mainly during parvovirus infections

  • Treatment of choice for idiopathic cases is immunosuppressive therapy with a calcineurin inhibitor (ie, cyclosporine)

  • Monotherapy with corticosteroids produce responses in 30–60% of cases, but relapses are common once corticosteroids are discontinued

  • Anti-CD20 monoclonal antibody (rituximab) is the treatment of choice in the setting of anti-erythropoietin antibodies

  • In patients with an associated lymphoproliferative disorder, treating the neoplasm should also treat the anemia

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