Successful major and minor surgery using factor VIII inhibitor bypassing activity in patients with haemophilia A and inhibitors

Abstract

Surgeries are being increasingly performed in patients with haemophilia A and high-titre inhibitors. Optimal bypassing agent regimens need further delineation. Data pertaining to surgeries from 1989 to 2004 at a single centre were retrospectively analysed. Patients received a standardized factor eight inhibitor bypassing activity (FEIBA) dose for both major and minor elective or emergency surgeries. The standard FEIBA dose was 70 U kg(-1) per infusion. FEIBA was infused at 9 and 1 h before and 8 h after operation. Infusions were routinely repeated every 8 h afterward. Haemostatic efficacy was assessed on the basis of blood loss, occurrence of haematoma and transfusion requirements. Seven adult patients underwent a total of 12 operations: 10 major and two minor. Ten procedures were elective. The median cumulative numbers of infusions and days of therapy were 46 and 17, respectively. Cumulative total FEIBA consumption was a median of 3185 U kg(-1). Observed blood losses, haematoma incidence and transfusion requirements were comparable to those expected for noncoagulopathic patients undergoing similar procedures. The only large haematoma occurred after a hip prosthesis operation and resolved under continuing FEIBA treatment. There were no cases of disseminated intravascular coagulation or other thromboembolic complications. FEIBA provides an effective and safe first-line peri- and postoperative haemostatic therapy for patients with haemophilia A and inhibitors, allowing both major and minor operations to be successfully performed.