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Von Willebrand factor-containing factor VIII concentrates and inhibitors in haemophilia A

Journal: Thrombosis and Haemostasis
ISSN: 0340-6245
Topic:

Theme Issue Ratnoff Symposium 2009

DOI: https://doi.org/10.1160/TH10-03-0151
Issue: 2010: 104/5 (Nov) pp. 863-1082
Pages: 931-940

Von Willebrand factor-containing factor VIII concentrates and inhibitors in haemophilia A

A critical literature review

M. Franchini (1), G. Lippi (2)

(1) Servizio di Immunoematologia e Trasfusione, Azienda Ospedaliero-Universitaria di Parma, Italy; (2) U.O. di Diagnostica Ematochimica, Azienda Ospedaliero-Universitaria di Parma, Italy

Keywords

FVIII, VWF, Inhibitors, haemophilia A, immune tolerance induction (ITI)

Summary

The development of inhibitors that neutralise the function of factor VIII (FVIII) is currently not only the most challenging complication associated with the treatment of haemophilia A but it also increases the disease-related morbidity as bleeding episodes do not respond to standard therapy. The main short-term goal of the treatment of inhibitor patients is to control bleeding episodes while the long-term one is to permanently eradicate the inhibitor by immune tolerance induction, particularly in the case of high-titer antibodies. Due to some in vitro studies and clinical observations, some investigators have suggested that FVIII concentrates containing von Willebrand factor (VWF) may be less immunogenic than high-purity or recombinant FVIII products. It has also been suggested that success rates for immune tolerance induction are higher when plasma-derived FVIII products are used. The currently available data from laboratory and clinical studies on the role of VWF in inhibitor development and eradication in haemophilia A is critically analysed in this review. As a result, we have not found definitive evidence supporting a role for product type on inhibitor incidence and inhibitor eradication in haemophilia A patients.

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