Inhibitor development

Jan Astermark; S. Lacroix-Desmazes; M. T. Reding

doi:10.1111/j.1365-2516.2008.01711.x

Inhibitor development

Jan Astermark, S. Lacroix-Desmazes, M. T. Reding

Medicine - Hematology, Oncology, Transplant

Research output: Contribution to journal › Review article › peer-review

42 Scopus citations

Abstract

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.

Original language	English (US)
Pages (from-to)	36-42
Number of pages	7
Journal	Haemophilia
Volume	14
Issue number	SUPPL. 3
DOIs	https://doi.org/10.1111/j.1365-2516.2008.01711.x
State	Published - Jul 2008

Keywords

Factor VIII
Haemophilia
Inhibitors
Risk factors
T-regulatory cells
Von Willebrand factor

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AU - Reding, M. T.

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AB - 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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KW - Haemophilia

KW - Inhibitors

KW - Risk factors

KW - T-regulatory cells

KW - Von Willebrand factor

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Inhibitor development

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