Factor VIII inhibitors: risk factors and methods for prevention and immune modulation.
Patients with hemophilia A are deficient in coagulation Factor VIII. This bleeding disorder can be treated with Factor VIII replacement therapy, but close to a third of patients will be immunized to the treatment and begin to form inhibitory antibodies known as "inhibitors". These inhibitors will render the treatment ineffective and represent the most severe complication in the treatment of hemophilia A. In this review, we highlight factors involved in inhibitor development and emphasize research being done to modulate the immune response to this life-saving therapy.
Zhang
Ai Hong
AH
Department of Surgery, and Center for Vascular and Inflammatory Diseases, University of Maryland School of Medicine, 800 West Baltimore Street, Room 319, Baltimore, MD 21201, USA.
Skupsky
Jonathan
J
Scott
David W
DW
D000818:Animals; D001323:Autoantibodies / Q000097:blood* / Q000276:immunology; D019774:Blood Coagulation Factor Inhibitors / Q000097:blood / Q000276:immunology; D005169:Factor VIII / Q000235:genetics / Q000276:immunology* / Q000627:therapeutic use; D006467:Hemophilia A / Q000276:immunology / Q000628:therapy; D006801:Humans; D007108:Immune Tolerance; D007518:Isoantibodies / Q000097:blood* / Q000276:immunology; D051379:Mice; D012307:Risk Factors
D001323:Autoantibodies; D019774:Blood Coagulation Factor Inhibitors; D007518:Isoantibodies; D005169:Factor VIII