Inhibitor development.
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.
Department for Coagulation Disorders, Malmö University Hospital, Malmö, Sweden. jan.astermark@med.lu.se
J
J
Astermark
S
S
Lacroix-Desmazes
M T
MT
Reding
D001323:Autoantibodies / Q000276:immunology / Q000378:metabolism; D015551:Autoimmunity / Q000276:immunology; D019774:Blood Coagulation Factor Inhibitors / Q000276:immunology*; D005169:Factor VIII / Q000276:immunology*; D015316:Genetic Therapy / Q000379:methods; D006467:Hemophilia A / Q000235:genetics* / Q000276:immunology; D006801:Humans; D011110:Polymorphism, Genetic / Q000235:genetics* / Q000276:immunology; D050378:T-Lymphocytes, Regulatory / Q000276:immunology / Q000378:metabolism; D014841:von Willebrand Factor / Q000276:immunology
D001323:Autoantibodies; D019774:Blood Coagulation Factor Inhibitors; D014841:von Willebrand Factor; D005169:Factor VIII