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Semin Thromb Hemost 2012; 38(07): 735-741
DOI: 10.1055/s-0032-1326779
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Acquired Hemophilia A

Kathryn E. Webert
1   Canadian Blood Services, Medical Office, Hamilton, Ontario, Canada
2   Department of Pathology and Molecular Medicine, McMaster University, Hamilton, Ontario, Canada
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Publikationsdatum:
01. September 2012 (online)

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Abstract

Acquired hemophilia A is a rare, autoimmune disorder that is caused by autoantibodies that act as inhibitors to factor VIII. It is characterized by severe, unexpected bleeding that may be life-threatening. The incidence of acquired hemophilia A is ∼ 0.2 to 1.48 cases per 1 million individuals per year. Acquired hemophilia A has been associated with several clinical conditions including pregnancy, autoimmune or collagen vascular disorders, malignancies, drugs, respiratory disorders, and infections. However, in ∼ 50% of cases, no disease association is determined. Acquired hemophilia A should be suspected when a patient with no previous personal or family history of bleeding, presents with bleeding and an unexplained prolonged activated partial thromboplastin time (APTT) and other common causes of a prolonged APTT are ruled out. The treatment of acquired hemophilia A has two main goals: (1) to treat and/or prevent bleeding complications and (2) to eradicate the inhibitor. The recommended agents to be used for the treatment or prevention of bleeding in patients with acquired hemophilia A are the bypassing agents. Patients should be treated initially with corticosteroids, either alone or in combination with cyclophosphamide, to eradicate the inhibitor.

Keywords

acquired hemophilia A - inhibitors - bleeding