How we treat a hemophilia A patient with a factor VIII inhibitor.
The most significant complication of treatment in patients with hemophilia A is the development of alloantibodies that inhibit factor VIII activity. In the presence of inhibitory antibodies, replacement of the missing clotting factor by infusion of factor VIII becomes less effective. Once replacement therapy is ineffective, acute management of bleeding requires agents that bypass factor VIII activity. Long-term management consists of eradicating the inhibitor through immune tolerance. Despite success in the treatment of acute bleeding and inhibitor eradication, there remains an inability to predict or prevent inhibitor formation. Ideally, prediction and ultimately prevention will come with an improved understanding of how patient-specific and treatment-related factors work together to influence anti-factor VIII antibody production.
Kempton
Christine L
CL
AFLAC Cancer Center and Blood Disorders Service and Winship Cancer Institute, Emory University, Atlanta, GA 30322, USA. ckempto@emory.edu
White
Gilbert C
GC
D005169:Factor VIII / Q000008:administration & dosage* / Q000037:antagonists & inhibitors / Q000276:immunology*; D006467:Hemophilia A / Q000188:drug therapy* / Q000453:epidemiology / Q000276:immunology*; D006801:Humans; D007108:Immune Tolerance; D007518:Isoantibodies / Q000276:immunology; D012307:Risk Factors
D007518:Isoantibodies; D005169:Factor VIII