Two Cases of Acquired Hemophilia A Successfully Treated with Oral Steroid or Danazol

Sun Young Park; Jin Seok Kim; Yuri Kim; In Hae Park; June-Won Cheong; Seung Tae Lee; Jee Sook Hahn; Yoo Hong Min

doi:10.5045/kjh.2005.40.1.58

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Park, Kim, Kim, Park, Cheong, Lee, Hahn, and Min: Two Cases of Acquired Hemophilia A Successfully Treated with Oral Steroid or Danazol

Case Report

Korean J Hematol 2005;40(1):58-63.

Published online: 21 March 2005

DOI: https://doi.org/10.5045/kjh.2005.40.1.58

Two Cases of Acquired Hemophilia A Successfully Treated with Oral Steroid or Danazol

Sun Young Park, M.D., Jin Seok Kim, M.D., Yuri Kim, M.D., In Hae Park, M.D., June-Won Cheong, M.D., Seung Tae Lee, M.D., Jee Sook Hahn, M.D., Yoo Hong Min, M.D.

Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea

Correspondence to: Jin Seok Kim, M.D. Department of Internal Medicine, Yonsei University College of Medicine 134 Sinchon-dong, Seodaemun-gu, Seoul 120-752, Korea Tel: +82-2-2228-1930, 1972 Fax: +82-2-393-6884 E-mail: hemakim@yumc.yonsei.ac.kr

Received 2 February 2005 Revised 3 March 2005 Accepted 11 March 2005

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Acquired Hemophilia A is a rare and considerably life-threatening coagulopathy, which is caused by the development of autoantibodies against factor VIII (FVIII) in non-hemophilic adults. Acquired FVIII inhibitors can be associated with diverse conditions, such as malignant disorders, medications, autoimmune diseases, postpartum states and others. These autoantibodies inhibit normal coagulation, had results in bleeding complications, which can contribute to mortality in a high percentages of cases. Effective control of the disorder can be achieved by prompt diagnosis and appropriate managements. Generally, the managements of acquired hemophilia A are aimed at treating the acute bleeding and eliminating inhibitors by immunosuppression. Although a range of treatment options exists for patients with acquired hemophilia A, there is no consensus with regard to the optimal therapies for this disorder. Herein, two cases, an 82-year-old man and a 78-year-old man who were successfully treated by steroid or danazol, which is a relatively mild immunosuppressive agent, are reported.

Keywords: Acquired hemophilia A, Factor VIII inhibitor, Immunosuppressive therapy

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Fig. 1.

Multiple petechiae were shown at bilateral upper (A) and lower extremities (B) in case 1 patient.

Fig. 2.

Markedly elevated geographic ulcerations were shown at distal esophagus on esophagogastroduodenoscopy in case 1 patient.

Fig. 3.

The changes of concentration of factor VIII inhibitor, levels of factor VIII activity and aPTT in case 1 patient treated with prednisolone (PL) and danazol.

Fig. 4.

The changes of concentration of factor VIII inhibitor, levels of factor VIII activity and aPTT in case 2 patient treated with prednisolone (PL).

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