Congenital haemophilia A with inhibitor treated with recombinant factor VIIa in an infusion pump

Streszczenie

Congenital haemophilia A is a rare disorder where low factor VIII activity is the result of gene
defect. Human coagulation factor VIII concentrate is used to prevent bleeding or for therapy.
The main therapy-related complication is development of an inhibitor against Factor VIII. Inhibitors
are alloantibodies which neutralize procoagulant activity of exogenous factor VIII during
replacement therapy. Bypassing agents are then the treatment of choice and can substitute
factor VIII in maintaining haemostasis. Recombinant factor VIIa and activated prothrombin
complex concentrates are of comparable effectiveness.
Significant numbers of haemophilia A patients with inhibitor are treated with recombinant
factor VIIa. So far, it has been administered in bolus injections every 2 to 12 hours. Infusion
pump is a promising alternative to bolus injections. Delivery of recombinant factor VIIa in an
infusion pump has been proved effective in terms of maintaining accurate drug levels as well
as minimizing human errors by reducing burden on staff.