Sequential combined bypassing therapy in haemophilia patients with high titer inhibitors: surgical experience.

Abstract

Sequential combination bypass therapy (SCBT) is an effective treatment option for haemophilia patients with inhibitors; however, its safety, efficacy, and cost have largely have yet to be systematically evaluated. To address this question, we retrospectively analyzed the medical records of 14 haemophilia patients with high titer inhibitors who underwent surgery. The patients with high inhibitors were treated with two SCBT regimens by optimizing doses of the recombinant activated factor VII (rFVIIa) and prothrombin complex concentrate (PCC). The effectiveness and safety of the two SCBT regimens were evaluated. In addition, rFVIIa and PCC factor consumption and costs were also compared. The median age of the 14 patients was 30.00 (27.25-42.75) years. They all underwent major surgeries, with 85.71% (12/14) was orthopedic surgeries related to hemophilic arthropathy. Four patients were treated using regimen 1 and ten with regimen 2. Results showed that regimen 2 exhibited a higher haemostatic efficiency (90% vs. 75% intraoperative and 90% vs. 50% postoperative) and a 28.0% reduction in economic costs (863,604.68 RMB vs. 621,756.62 RMB). All patients after surgery had no prothrombin time extension, 7.14% had fibrinogen and platelet count decreases, and 57.14% had D-dimer increases that returned to baseline within 5-7 days after SCBT. The study shows that regimen 2 as an optimized SCBT regimen is an efficient approach to secure haemostasis for haemophilia patients with high titer inhibitors in the perioperative period, rather than regimen 1. The findings can help design future clinical studies and provide more reliable data and implementation advice.