Relapsing pure red cell aplasia associated with B-cell chronic lymphocytic leukemia successfully treated by intravenous immunoglobulin concentrate.

Pure red cell aplasia (PRCA) is a rare syndrome characterized by a normochromic normocytic anemia and the absence of mature erythroid precursors in an otherwise normocellular bone marrow. Acquired PRCA has been associated with autoimmune, viral or neoplastic conditions. We report the case of a patient with B-cell chronic lymphocytic leukemia (B-CLL) and PRCA. Conventional antileukemic treatment had no effect on the PRCA, while the B-CLL showed partial remission according to the International Workshop on Chronic Lymphocytic Leukemia response criteria. The patient was treated with cyclosporin A that resulted in complete response of the PRCA lasting 12 months. PRCA relapse, suggested by a hemoglobin decrease and by bone marrow biopsy, did not respond to prednisone and cyclophosphamide treatment. Fludarabine treatment was started in July 1999; the B-CLL remained stable according to the International Workshop on Chronic Lymphocytic Leukemia response criteria, the PRCA did not improve. In October 1999, because of intercurrent pneumonia, the patient was treated with intravenous immunoglobulin (IVIG) concentrate. This treatment resulted in total resolution of the pneumonia and a complete response of the PRCA. Therefore the patient remained on monthly IVIG. In view of the known efficacy of IVIG in the treatment of PRCA induced by parvovirus B19 and since serial polymerase chain reaction determinations of parvovirus B19 were repeatedly negative in our subject, we hypothesize that the IVIG per se may have a therapeutic effect on PRCA. The present case suggests that IVIG may be of benefit for PRCA patients even if the disease is unrelated to parvovirus B19 infection.