Bohn Jan-Paul, Fiala Anna, Bachmann Sebastian, Irsara Christian, Wolf Dominik, Feistritzer Clemens
Department of Internal Medicine V, Hematology and Oncology, Medical University of Innsbruck, Anichstrasse 35, A-6020 Innsbruck, Austria.
Department of Anesthesiology and Critical Care Medicine, Medical University of Innsbruck, Innsbruck, Austria.
Ther Adv Hematol. 2022 Jul 26;13:20406207221104595. doi: 10.1177/20406207221104595. eCollection 2022.
The introduction of extended factor IX (FIX) products has significantly facilitated the treatment of hemophilia B patients. However, optimal perioperative management remains a topic of hot debate, particularly in surgeries with high bleeding risk. For the first time, we report here a patient with mild hemophilia B and degenerative aneurysms of aortic root and ascending aorta undergoing elective Bentall's operation with full cardiopulmonary bypass, who was successfully managed with eftrenonacog alfa (Alprolix), a recombinant FIX Fc fusion protein (rFIXFc). rFIXFc could safely be monitored using the Pathromtin SL aPTT-reagent. No significant bleeding was noted intraoperatively despite systemic heparinization as well as postoperatively. Higher doses of rFIXFc were inevitable to reach target FIX levels intraoperatively, whereas in the post-surgery setting stable FIX concentrations were maintained with only few rFIXFc injections facilitating fast wound healing and remobilization of the patient.
延长型凝血因子IX（FIX）产品的引入显著促进了B型血友病患者的治疗。然而，围手术期的最佳管理仍然是一个激烈争论的话题，尤其是在出血风险高的手术中。我们首次在此报告一名患有轻度B型血友病以及主动脉根部和升主动脉退行性动脉瘤的患者，该患者接受了择期Bentall手术并进行了全心肺转流，使用重组FIX Fc融合蛋白（rFIXFc）eftrenonacog alfa（Alprolix）成功进行了管理。rFIXFc可以使用Pathromtin SL aPTT试剂进行安全监测。尽管术中进行了全身肝素化以及术后，均未观察到明显出血。术中不可避免地需要更高剂量的rFIXFc才能达到目标FIX水平，而在术后仅需注射少量rFIXFc就能维持稳定的FIX浓度，这有利于患者伤口快速愈合和活动恢复。
