Porcine factor VIII in the treatment of high-titre inhibitor patients

Development of an inhibitor against factor VIII (FVIII) is an important complication of haemophilia. It occurs in approximately 25-30% of patients with haemophilia A [1]. FVIII inhibitors may also occur as autoantibodies. The latter occur in non-haemophiliacs and, although rare (occurring in approximately one per million of the population), are frequently associated with life-threatening bleeding. Inhibitors are considered low level if they are < 5 Bethesda Units (BU) or high level if they are > 10 BU. The former usually remain low and rarely give anamnestic response, the latter do so frequently. Despite various approaches to their management, the presence of inhibitors remains a major cause of morbidity and mortality. The effectiveness of porcine FVIII (pFVIII) in treating patients with both auto- and alloantibodies to FVIII has been well demonstrated when adequate circulating levels of FVIII are obtained [2--10]. However, pFVIII therapy may give rise to antibodies to the pFVIII and the utility of this treatment in the presence of high levels of porcine antibody is less well recognized and understood. Nonetheless, pFVIII under these circumstances may be useful in a select group of patients where management is difficult.