Recommendations for the treatment of factor VIII inhibitors: from the UK Haemophilia Centre Directors' Organisation Inhibitor Working Party.
A strategy is described for the initial detection, management and elimination of factor VIII inhibitors arising in patients with congenital and acquired haemophilia A. It is suggested that children with severe haemophilia A should be screened every 3 months up to the age of 10 years for inhibitors using the Bethesda method. Factor VIII inhibitors arising in these patients should be abolished using immune-tolerance induction wherever possible. Such regimes should be started as early as possible, preferably when the inhibitor titre is < 10 Bethesda Units (BU)/ml, and should not be interrupted. High-intensity regimes are recommended for patients whose inhibitors exceed 10 BU. Autoantibodies to factor VIII giving rise to acquired haemophilia should be abolished using high-dose immunoglobulin or conventional immunosuppression. The choice of haemostatic agent for the treatment of severe bleeding should be based upon the clinical circumstances and the current inhibitor value, measured using both human and porcine factor VIII in the Bethesda assay. The past anamnestic response should also be considered when choosing treatment for minor bleeding episodes.
Hay
C R
CR
University Department of Haematology, Manchester Royal Infirmary, UK.
Colvin
B T
BT
Ludlam
C A
CA
Hill
F G
FG
Preston
F E
FE
D001779:Blood Coagulation Factors / Q000627:therapeutic use; D016913:Blood Component Transfusion / Q000191:economics; D005169:Factor VIII / Q000037:antagonists & inhibitors* / Q000627:therapeutic use; D015942:Factor VIIa / Q000627:therapeutic use; D006467:Hemophilia A / Q000150:complications / Q000628:therapy*; D006470:Hemorrhage / Q000150:complications / Q000628:therapy; D006801:Humans; D011994:Recombinant Proteins / Q000627:therapeutic use
D001779:Blood Coagulation Factors; D011994:Recombinant Proteins; D005169:Factor VIII; C065655:anti-inhibitor coagulant complex; D015942:Factor VIIa