Prophylaxis in Congenital Hemophilia with Inhibitors: The Role of Recombinant Activated Factor VII
Massimo Franchini1
, Franco Manzato2
, Gian Luca Salvagno3
, Martina Montagnana3
, Marco Zaffanello4
, Giuseppe Lippi5
1Servizio di Immunoematologia e Medicina Trasfusionale, Dipartimento di Patologia e Medicina di Laboratorio, Azienda Ospedaliero-Universitaria di Parma, Parma
2Laboratorio di Patologia Clinica, Ospedale “Carlo Poma”, Mantova
3Sezione di Chimica Clinica, Dipartimento di Scienze Biomediche e Morfologiche, Università di Verona, Verona
4Dipartimento Materno-Infantile e di Biologia Genetica; Verona
5Unità Operativa di Diagnostica Ematochimica, Dipartimento di Patologia e Medicina di Laboratorio, Azienda Ospedaliero-Universitaria di Parma, Parma; Italy
The development of inhibitors against therapeutically administered factors VIII or IX is actually the most challenging complication of hemophilia patients with inhibitors. The introduction of bypassing agents (i.e., activated prothrombin complex concentrates and recombinant activated factor VII [rFVIIa]) has dramatically improved the management of bleeding episodes in such patients. Over the last decade, there have been increasing reports on the ability of bypassing agents to prevent surgical, joint, or other bleeds in inhibitor patients. The published data on the use of rFVIIa as a prophylactic treatment in hemophilia patients with inhibitors are reviewed in this article.
KEYWORDS
rFVIIa - prophylaxis - surgery - bleeding
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