PURE RED-CELL APLASIA ASSOCIATED WITH ANGIOIMMUNOBLASTIC LYMPHADENOPATHY WITH DYSPROTEINEMIA

Angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) can best be described as a disorder of T-cells resulting in amplification of t he B-cell response and clinical symptoms of lymphadenopathy, fever, he patosplenomegaly, and a variety of blood abnormalities. Pure red cell aplasia (PRCA), an autoimmune disorder resulting in selective aplasia of the erythroid series, has only rarely been associated with AILD. He rein we report three cases of AILD and PRCA. Serum from one patient wa s available for study and contained a dose-dependent inhibitor of the CFU-E but not CFU-GM cultures from normal bone marrow. This activity w as found in the globulin fraction after ammonium sulfate precipitation . Patients with AILD are known to make antibodies to many autologous e pitopes, and the most well-characterized mechanism of PRCA involves an tibodies to red cell precursors. Our serum data are consistent with th e hypothesis that such an antibody existed in our patient. Aggressive treatment of these patients resulted in transient improvement in two; however, all three died without achieving a durable complete remission with two dying of infectious complications. (C) 1994 Wiley-Liss, Inc.