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Pure red cell aplasia (PRCA): Response of three patients of
cyclophosphamide and/or antilymphocyte globulin (ALG) and demonstration of
two types of serum IgG inhibitors to erythropoiesis
A Marmont, C Peschle, M Sanguineti and M Condorelli
Three cases of adult pure red cell aplasia (PRCA) ARE REPORTED. All
patients proved refractory to various combinations of androgens and
corticosteroids. The first case, harboring a thymoma, showed a complete
clinical remission following cyclophosphamide therapy. The second and third
responded similarly to either a combined cyclophosphamide + antilymphocyte
globulin (ALG) treatment or to ALG administration preceded by a small
dosage of cyclophosphamide, which had proved ineffective when administered
alone. Serum IgG inhibitors to erythropoiesis were demonstrated in all
cases by means of in vivo and/or in vitro techniques. The inhibitor(s),
although directed against the erythroid marrow in both the first and third
patients (PRCA type A), apparently functioned as an antibody to circulating
erythropoientin (Ep) in the second case (PRCA type B). The inhibitor(s) was
always absent in postremission samples. Additionally, experimental models
for both types of human PRCA were established in normal rodents. The
present studies support the contention that adult PRCA is an autoimmune
disease. The therapeutic role of cytotoxic-immunodepressive agents in PRCA
patients is confirmed. It is emphasized that ALG may represent an
additional therapeutic tool in cases resistant to cyclophosphamide and/or
steroids. In addition, cyclophosphamide proved effective in a patient
harboring a thymoma not amenable to surgery. Finally, it is postulated that
IgG serum autoantibodies, directed against either an early erythroid
precursor (PRCA type A) or, more rarely, circulating Ep (PRCA type B), play
a major role in the pathogenesis of the disease.
Volume 45,
Issue 2,
pp. 247-261,
02/01/1975
Copyright © 1975 by The American Society of Hematology
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