Alloanti-VIII inhibitor
Definition:
Antibodies that specifically inhibit factor VIII and are produced by patient in response to injected factor VIII. (These show normal type I kinetics)
Incidence:
Seen in the following patients:
Hemophilia A – (20 – 25% of patients)
Detection:
aPTT that is prolonged and not corrected when mixing studies are performed and if the incubation time is increased to 2 hours at 37oC it remains prolonged
Therapy:
Corticosteroids to suppress immune system
Porcine factor VIII
Plasma exchange if severe