Inhibitor Development

Authors


  • The authors stated that they had no interests which might be perceived as posing a conflict or bias.

Jan Astermark, MD, PhD, Associate Professor, Department for Coagulation Disorders, Malmö University Hospital, SE-205 02 Malmö, Sweden.
Tel.: +46 40 332392; fax: +46 40 336255;
e-mail: jan.astermark@med.lu.se

Abstract

Summary.  The immune response to factor VIII and the development of inhibitory antibodies is a complex multi-factorial process involving a variety of immune regulatory genes and cells, several of which have the potential to determine risk. A better understanding of the mechanisms involved will increase the likelihood of development of new therapeutic options for patients with hemophilia. This review summarizes genetic and non-genetic risk factors currently under evaluation, and the potential modulative effect of the von Willebrand factor on factor VIII immuno- and antigenicity. In addition, the role of T-regulatory cells in the pathogenicity of inhibitors will be discussed.

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