Therapy in Hemophilia A with Inhibitors
Give factor VIII concentrate
Therapy for low responders:
If no elevation of VIII:
40 U/Kg will neutralize 1 Bethesda unit/mL for 2 – 3 days
If still there is no response and patient is bleeding:
Give injections of factor VIIa – Why?
Switch to porcine factor VIII
VIIa will activate the common pathway (X to Xa) and thus initiate clot formation.
Try to initiate tolerance:
Give prednisone or cytoxan
Try to suppress immune system:
Give high doses of VIII for months or years