Treatment of refractory pure red cell aplasia with cyclosporine A: disappearance of IgG inhibitor associated with clinical response.
Remissions were obtained in 6/9 evaluable patients with pure red cell asplasia (PRCA) refractory to other immunosuppressive agents who were treated with cyclosporine A (CsA). Four of these patients have remained in continuous remission off all treatment for 4-19 months. Another patient who stopped CsA abruptly relapsed, but responded to reinstitution of therapy. The sixth patient died of a cerebrovascular accident while in remission on a low dose of CsA. Acute side effects were minimal and were responsive to dose reduction. One patient developed a lymphoma while in an unmaintained remission, and one patient who did not respond to CsA was found to have a lymphoma approximately a year after stopping treatment. In vitro studies of autologous erythroid progenitors in a patient with an IgG inhibitor of erythropoiesis showed a reduction of autoantibody associated with the response to CsA. The antigen to which this inhibitor is directed was expressed only during the marrow erythroid burst-forming unit (BFU-E) period of erythroid differentiation. CsA can induce sustained remissions in cases of PRCA refractory to other multiple agents, and these remissions may be associated with a reduction in autoantibody to erythroid progenitor cells. Further studies of patients with PRCA who respond to CsA may lead to an improved understanding of this disorder.
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Department of Medicine, Department of Veterans Affairs Medical Center, Nashville, TN 37212.
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Krantz
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D000293:Adolescent; D000328:Adult; D000368:Aged; D001323:Autoantibodies / Q000276:immunology*; D002478:Cells, Cultured; D003524:Cyclosporins / Q000009:adverse effects / Q000627:therapeutic use*; D015672:Erythroid Precursor Cells / Q000276:immunology; D004920:Erythropoiesis / Q000276:immunology; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology* / Q000302:isolation & purification; D008223:Lymphoma / Q000139:chemically induced; D008297:Male; D008875:Middle Aged; D012010:Red-Cell Aplasia, Pure / Q000188:drug therapy*
D001323:Autoantibodies; D003524:Cyclosporins; D007074:Immunoglobulin G