Rener Karla, Anžej Doma Saša, Fink Martina, Podgornik Helena, Preložnik Zupan Irena
Department of Hematology, University Medical Center Ljubljana, Zaloška cesta 7, 1000 Ljubljana, Slovenia.
Faculty of Medicine, University of Ljubljana, Vrazov trg 2, 1000 Ljubljana, Slovenia.
Hematol Rep. 2023 Nov 1;15(4):597-607. doi: 10.3390/hematolrep15040062.
Prophylactic treatment with emicizumab has become an important and effective bleeding prevention for people with hemophilia A (PwHA). Perioperative management of PwHA using emicizumab prophylaxis is still challenging due to a lack of experience. Medical records of perioperative management and outcomes were reviewed, and data were collected for adult PwHA receiving emicizumab and undergoing surgical procedures between August 2019 and July 2022 at the University Medical Center Ljubljana. Twelve surgical procedures were performed in eight PwHA (one with FVIII inhibitors) while on emicizumab prophylaxis. Three minor procedures included cataract surgery, cystoscopic lithotripsy, and percutaneous coronary intervention. Nine major surgeries included four osteosyntheses, necrectomy of chronic osteomyelitis with new ankle arthrodesis, two below-knee amputations, total knee replacement, and placement of ventriculostomy after a spontaneous intraventricular hemorrhage. No major bleeds, thrombotic events or deaths, or new inhibitors appeared. Our real-world experience demonstrates that minor and major surgeries can be performed safely in PwHA on emicizumab prophylaxis. Additional data are needed to optimize dosing/duration of additional hemostatic agents in diverse invasive procedures and complex clinical situations.
艾美赛珠单抗预防性治疗已成为甲型血友病患者(PwHA)预防出血的重要且有效方法。由于经验不足,使用艾美赛珠单抗进行预防性治疗的PwHA围手术期管理仍具有挑战性。回顾了围手术期管理及结果的病历,并收集了2019年8月至2022年7月在卢布尔雅那大学医学中心接受艾美赛珠单抗治疗并接受外科手术的成年PwHA的数据。8例PwHA(1例有FVIII抑制剂)在接受艾美赛珠单抗预防性治疗期间进行了12台外科手术。3例小手术包括白内障手术、膀胱镜碎石术和经皮冠状动脉介入治疗。9例大手术包括4例骨固定术、慢性骨髓炎病灶清除术并伴有新的踝关节融合术、2例膝下截肢术、全膝关节置换术以及自发性脑室内出血后放置脑室造瘘管。未出现大出血、血栓事件或死亡,也未出现新的抑制剂。我们的真实世界经验表明,接受艾美赛珠单抗预防性治疗的PwHA可以安全地进行小手术和大手术。需要更多数据来优化在各种侵入性手术和复杂临床情况下额外止血剂的剂量/持续时间。