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

Massimo Franchini; Giuseppe Lippi

doi:10.1160/TH10-03-0151

Thrombosis and Haemostasis, Table of Contents

Thromb Haemost 2010; 104(05): 931-940
DOI: 10.1160/TH10-03-0151

Review Article

Schattauer GmbH

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

A critical literature review

Massimo Franchini

¹Servizio di Immunoematologia e Trasfusione, Azienda Ospedaliero-Universitaria di Parma, Italy

,

Giuseppe Lippi

²U.O. di Diagnostica Ematochimica, Azienda Ospedaliero-Universitaria di Parma, Italy

› Author Affiliations

Abstract

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.

Keywords

FVIII - inhibitors - haemophilia A - immune tolerance induction (ITI) - VWF

Full Text

References

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