Korean J Hematol.  2005 Mar;40(1):58-63. 10.5045/kjh.2005.40.1.58.

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

Affiliations
  • 1Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea. hemakim@yumc.yonsei.ac.kr

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.

Keyword

Acquired hemophilia A; Factor VIII inhibitor; Immunosuppressive therapy

MeSH Terms

Adult
Aged
Aged, 80 and over
Autoantibodies
Autoimmune Diseases
Consensus
Danazol*
Diagnosis
Factor VIII
Hemophilia A*
Hemorrhage
Humans
Immunosuppression
Mortality
Postpartum Period
Autoantibodies
Danazol
Factor VIII

Figure

  • 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).


Cited by  1 articles

Post-operative Bleeding due to Acquired Hemophilia Successfully Treated with Recombinant Factor VIIa: Case Report
Naria Lee, Seong Hoon Yoon, Won Lim, Mi Hyun Kim, Hyo Jeong Kim, Seon Kyeong Kim, Jin Sup Park, Young Mi Seol, Moo Kon Song, Young Jin Choi, Joo Seop Chung, Goon Jae Cho
Korean J Hematol. 2009;44(3):163-167.    doi: 10.5045/kjh.2009.44.3.163.


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