Multicenter Retrospective Study on the Utilization of FEIBA in France in Patients with Factor VIII and Factor IX Inhibitors

Abstract

SummaryFactor VIII or factor IX replacement is frequently impossible in inhibitor-developing hemophiliacs, because of the level of the inhibitor titer. Activated prothrombin complex concentrates are one of the available options to treat the bleeding episodes in such patients. However, the efficacy of these products and the associated thrombogenic risk, particularly in prolonged administration such as employed during surgeries, are important concerns for hemophilia care providers. We performed a multicenter retrospective study to evaluate the use of FEIBA (Factor Eight Bypassing Activity) in France, and data is presented on 433 bleeding episodes, including surgical procedures, concerning 60 patients from 15 hemophilia centers.The efficacy was judged as good or excellent in 352 episodes (81.3%), poor in 73 episodes (16.9%) and non-existent in 8 episodes (1.8%). Minor and major surgical procedures were successfully performed using FEIBA as a second-line therapy after human or porcine factor VIII, and in some occasions FEIBA was utilized as the only substitution product. The tolerance was assessed as good in 428 episodes (98.8%), but in 5 cases adverse effects were reported. Only 3 patients out of 52 regularly evaluated (5.8%) were HIV-seropositive, and for two of them the seroconversion occurred prior to the first use of FEIBA. In contrast, 80.4% of the patients were HCV-seropositive. An anamnestic response after the administration of FEIBA was noted in 31.5% of cases. This study points out the main features of the use of FEIBA in France, and particularly the low HIV seroprevalence in the patients treated. The good efficacy and the excellent tolerance still confer to this product a place to consider in the therapeutic options for the treatment of inhibitor-developing hemophiliacs or in acquired hemophilia.

Prevention of bleeds in hemophilia patients with inhibitors: Emerging data and clinical direction

“…FEIBA has been evaluated in two published clinical trials involving a total of 75 patients and 583 bleeding episodes [24,25]. In both of these trials, FEIBA was well tolerated and found to be superior to PCCs in controlling bleeding episodes and improving joint mobility.…”

“…A number of published reports have described successful major surgeries for which only aPCCs or PCCs were used for prevention of bleeding, both intraoperatively and post-operatively [25,[40][41][42]. Numerous additional reports indicate that late post-surgical prophylaxis with aPCCs begun after the fifth to seventh post-operative day were also effective in preventing bleeding, even in patients undergoing physical therapy following major joint surgeries.…”

Successful use of recombinant factor VIIa for emergency fasciotomy in a patient with hemophilia A and high-titer inhibitor unresponsive to factor VIII inhibitor bypassing activity

“…Multiple studies with these products document efficacy of approximately 80% with either choice [5,[10][11][12]. However, hemostasis is not assured with any one bleeding episode with any one agent.…”

Successful self‐infusion of activated prothrombin complex concentrate for prophylaxis in a child with a factor VIII inhibitor

“…FVIII inhibitor bypassing activity (FEIBA VH [anti-inhibitor coagulant complex]) has been one of the most widely used APCC for the management of congenital or acquired hemophilia with high-titer inhibitors [2][3][4][5]. FEIBA also has been used for prophylaxis during immune tolerance induction [6].…”

“…To determine the safety and efficacy of PCC and APCC for prophylaxis, long-term observations are needed for sufficient number of patients. Negrier et al [4] suggested that the hemostatic efficacy of FEIBA was not compromised by the occurrence of anamnestic responses. This report documents successful home-based prophylaxis of FEIBA in a 14-year-old Japanese boy with severe hemophilia A and a high-titer inhibitor.…”