Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + Download Section PDF Listen ++ For further information, see CMDT Part 13-08: Pure Red Cell Aplasia + Key Features Download Section PDF Listen +++ ++ 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 Download Section PDF Listen +++ ++ 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 Download Section PDF Listen +++ ++ 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 Download Section PDF Listen +++ ++ 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 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