Prevention of bleeding in patients with haemophilia A complicated by factor VIII inhibitors

Abstract

The development of factor VIII inhibitor alloantibodies is nowadays the most important complication of the treatment of severe haemophilia A. The inhibitors occur in approximately 30% of patients with severe haemophilia A and render the treatment of bleeding episodes more difficult. The ultimate goal of treatment for patients with inhibitory antibodies should be to permanently eradicate the inhibitor by immune tolerance induction therapy (ITI). However, ITI procedures fail in 20–40% patients. In these patients, in those waiting for ITI to start, as well as in those undergoing ITI, acute bleeding episodes are generally managed by preparations containing activated coagulation factors: recombinant activated factor VII (rFVIIa) or activated prothrombin complex concentrate (aPCC). Results from recent studies clearly demonstrate that prophylactic administrations of rFVIIa and aPCC are highly effective in decreasing the frequency of bleeding episodes in haemophiliacs with inhibitors. This article discusses the role of rFVIIa and aPCC in the prevention of bleeding in haemophilia A complicated by factor VIII inhibitor.
Hematologia 2011; 2, 4: 311–317