Inhibitor development in haemophilia A: the role of von Willebrand factor/factor VIII concentrates.
The presence of inhibitors that neutralize the function of factor VIII (FVIII) decreases the haemostatic efficacy of replacement clotting factor concentrate and increases morbidity among patients with haemophilia A. Certain genetic and environmental variables have been linked to a higher incidence of inhibitors. Conversely, the presence of von Willebrand factor (VWF) in some plasma-derived FVIII products may provide some measure of protection against inhibitor development, although the evidence is not conclusive. Clinical trials are needed to resolve this issue and determine the appropriate role of VWF-containing FVIII concentrates in the treatment of haemophilia A patients.
Goudemand
J
J
Haematology Department, Lille University Hospital, Lille, France. j-goudemand@chru-lille.fr
D003029:Coagulants / Q000627:therapeutic use; D005169:Factor VIII / Q000276:immunology* / Q000627:therapeutic use; D006467:Hemophilia A / Q000188:drug therapy / Q000276:immunology*; D006490:Hemostatics / Q000627:therapeutic use*; D006801:Humans; D007518:Isoantibodies / Q000276:immunology*; D012306:Risk; D014841:von Willebrand Factor / Q000627:therapeutic use*
D003029:Coagulants; D006490:Hemostatics; D007518:Isoantibodies; D014841:von Willebrand Factor; C078147:F8 protein, human; D005169:Factor VIII