The Present Status of Treatment of Autoimmune Hemolytic Anemia with ACTH and Cortisone

Author:

DAMESHEK WILLIAM1,KOMNINOS ZACHARIAS D.1,DÉSY LOUISE1

Affiliation:

1. Ziskind Laboratories (Hematology section) of the Joseph H. Pratt and the New England Center Hospitals and the Department of Medicine, Tufts College Medical School.

Abstract

Abstract Forty-three patients with autoimmune hemolytic anemia (both "idiopathic" and "symptomatic") were treated with ACTH, Cortisone, or Compound F. In more recent cases not reported here, Prednisone (Meticorten) was used. Intensive and presumably adequate hormonal therapy resulted in sustained and complete clinical and hematologic responses in 65 per cent of the cases. Complete and sustained remissions were more commonly obtained in the "idiopathic" group (80 per cent remissions) than in the "symptomatic" variety. In 28 per cent of the cases, although the response was definite, it was not so striking. Complete failure was obtained in 3 cases (7%) of this series. When therapy was discontinued, following a remission, some degree of relapse developed in approximately two-thirds of the cases. In the remaining one-third, full clinical and hematologic remission continued well after discontinuance of therapy, although the persistently positive Coombs test indicated the possibility of future recurrence. Splenectomy was performed as the final therapeutic maneuver in 9 of the 43 cases and resulted in complete remissions in 6 without the further use of steroid hormones. In two cases splenectomy was followed by the development of outspoken disseminated lupus, previously undiscovered and presumably latent. It is apparent from these studies that the steroid hormones in adequate dosage represent a remarkably effective form of therapy for at least the initial control of most cases of autoimmune hemolytic anemia. Their use for a lengthy period is often followed by a lasting remission, and the end-results of continued steroid therapy, either used alone or in some cases combined with later splenectomy, are definitely better than with splenectomy alone. Thus, control of this rather unpredictable disease has become a distinct possibility in most cases, and its management simply a matter of varying the dosage of steroid hormone.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

Cited by 48 articles. 订阅此论文施引文献 订阅此论文施引文献,注册后可以免费订阅5篇论文的施引文献,订阅后可以查看论文全部施引文献

1. Characterization of direct antiglobulin test-negative autoimmune hemolytic anemia: A study of 154 cases;American Journal of Hematology;2012-11-21

2. Autoimmune Complications in Chronic Lymphocytic Leukaemia (CLL);Best Practice & Research Clinical Haematology;2010-03

3. Primary (Idiopathic) Auto-immune Haemolytic Anaemia;Acta Medica Scandinavica;2009-04-24

4. A Case of Paroxysmal Nocturnal Haemoglobinuria Splenectomized Twice;Acta Medica Scandinavica;2009-04-24

5. GLUCOCORTICOIDS IN HAEMATOLOGY;Acta Medica Scandinavica;2009-04-24

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