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Blood, 15 July 2008, Vol. 112, No. 2, pp. 250-255. Prepublished online as a Blood First Edition Paper on May 7, 2008; DOI 10.1182/blood-2008-03-143586. This Article Full Text Full Text (PDF) All Versions of this Article: blood-2008-03-143586v1 112/2/250    most recent Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via CrossRef Google Scholar Articles by Franchini, M. Articles by Lippi, G. PubMed PubMed Citation Articles by Franchini, M. Articles by Lippi, G. Related Collections Hemostasis, Thrombosis, and Vascular Biology How I Treat Free Research Articles Clinical Trials and Observations Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  HOW I TREAT Acquired factor VIII inhibitors Massimo Franchini1, and Giuseppe Lippi2 1 Servizio di Immunoematologia e Trasfusione, Azienda Ospedaliera Universitaria di Parma, Parma; and 2 Sezione di Chimica Clinica, Università di Verona, Verona, Italy 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. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this?

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