Department of Anaesthesiology, RWTH Aachen University Hospital, Pauwelsstraße 30, 52074, Aachen, Germany.
Department of Surgery, RWTH Aachen University Hospital, Aachen, Germany.
Anaesthesiologie. 2023 Dec;72(12):883-886. doi: 10.1007/s00101-023-01320-1. Epub 2023 Aug 10.
In patients with severe hemophilia A prolonged bleeding may occur even in cases of minor trauma or surgery.
To investigate the feasibility and efficacy of a recombinant extended half-life (EHL) FVIII concentrate for perioperative bleeding management in a patient with severe hemophilia A undergoing liver transplantation.
Prior to transplantation FVIII activity and perioperatively required FVIII supply were estimated. In an individualized approach efmoroctocog alfa was supplemented if the intrinsic clotting time in the thrombelastometry was > 170 s.
The patient perioperatively received a total of 28,000 IU efmoroctocog alfa. No signs of hemorrhage or complications were detected and no further intervention was necessary.
The present case demonstrates that the use of an EHL FVIII is suitable for a successful perioperative bleeding control even in hemophilia patients at a high bleeding risk during major surgery. Due to the EHL constant FVIII levels could be achieved with relatively few injections. In order to confirm the obtained results, more real-world data in different operative settings are essential. Further research is needed on the use of thrombelastometry to guide substitution of factor VIII perioperatively.
在重型血友病患者中，即使是轻微创伤或手术也可能导致长时间出血。
研究一种重组延长半衰期（EHL）FVIII 浓缩物在接受肝移植的重型血友病 A 患者围手术期出血管理中的可行性和疗效。
在移植前，估计 FVIII 活性和围手术期所需的 FVIII 供应。如果血栓弹性描记术中的固有凝血时间>170s，则采用个体化方法补充 efmoroctocog alfa。
患者围手术期共接受了 28000IU efmoroctocog alfa。未发现出血迹象或并发症，无需进一步干预。
本病例表明，即使在大手术期间出血风险较高的情况下，使用 EHL FVIII 也适合成功的围手术期出血控制。由于 EHL，FVIII 水平相对稳定，只需较少的注射次数即可达到。为了证实获得的结果，在不同的手术环境中需要更多的真实世界数据。还需要进一步研究血栓弹性描记术在围手术期因子 VIII 替代中的应用。
