Blood Journal
Leading the way in experimental and clinical research in hematology

Acquired factor VIII inhibitors

  1. Massimo Franchini1 and
  2. Giuseppe Lippi2
  1. 1Servizio di Immunoematologia e Trasfusione, Azienda Ospedaliera Universitaria di Parma, Parma; and
  2. 2Sezione di Chimica Clinica, Università di Verona, Verona, Italy
This article has an Erratum 113(21):5368

Abstract

Acquired hemophilia A is a rare bleeding diathesis caused by autoantibodies directed against clotting factor VIII and associated with an increased morbidity and mortality. This autoimmune disorder most commonly occurs in the elderly. Although it may be associated with several underlying pathologies, up to 50% of reported cases remain idiopathic. In contrast with congenital hemophilia, which is commonly characterized by hemarthroses, hemorrhages in patients with acquired hemophilia involve most frequently soft tissues. The 2 treatment priorities are to arrest the acute bleeding and to eradicate the factor VIII autoantibody. Acute bleeding episodes in patients with low-titer inhibitors can be treated using human factor VIII concentrates, whereas factor VIII bypassing agents, such as activated prothrombin complex concentrates or recombinant activated factor VII, are effective for the treatment of those with high-titer inhibitors. An analysis of the literature shows that the most effective first-line treatment for the eradication of factor VIII autoantibodies is the combination of steroids and cyclophosphamide. However, there is increasing evidence on the effectiveness of other treatment approaches, such as immune tolerance regimens and rituximab. If confirmed by large controlled studies, these innovative therapies might become a valid option for long-term eradication of factor VIII inhibitors.

  • Submitted March 15, 2008.
  • Accepted April 18, 2008.
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