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Pure red cell aplasia associated with angioimmunoblastic lymphadenopathy with dysproteinemia
Author(s) -
Lynch James W.,
Elfenbein Gerald J.,
Noyes Ward D.,
Braylan Raul C.,
Gross Mary Ann,
Weiner Roy S.
Publication year - 1994
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.2830460203
Subject(s) - pure red cell aplasia , hepatosplenomegaly , medicine , antibody , immunology , bone marrow , aplasia , globulin , pathology , disease
Angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) can best be described as a disorder of T‐cells resulting in amplification of the B‐cell response and clinical symptoms of lymphadenopathy, fever, hepatosplenomegaly, 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. Herein we report three cases of AILD and PRCA. Serum from one patient was available for study and contained a dose‐dependent inhibitor of the CFU‐E but not CFU‐GM cultures from normal bone marrow. This activity was found in the globulin fraction after ammonium sulfate precipitation. Patients with AILD are known to make antibodies to many autologous epitopes, and the most well‐characterized mechanism of PRCA involves antibodies to red cell precursors. Our serum data are consistent with the 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. © 1994 Wiley‐Liss, Inc.