Emicizumab预防的A型血友病患者侵入性治疗的管理和结果:单中心经验

Karla Rener, Saša Anžej Doma, Martina Fink, Helena Podgornik, Irena Preložnik Zupan
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引用次数: 0

摘要

emicizumab预防性治疗已成为血友病A (PwHA)患者重要而有效的出血预防方法。由于缺乏经验,使用emicizumab预防PwHA的围手术期管理仍然具有挑战性。回顾了2019年8月至2022年7月在卢布尔雅那大学医学中心接受emicizumab并接受外科手术的成年PwHA围手术期管理和结果的医疗记录,并收集了数据。8例PwHA患者(1例使用FVIII抑制剂)在emicizumab预防期间进行了12次外科手术。三种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次主要手术包括4次植骨、慢性骨髓炎伴新踝关节融合术的坏死切除、2次膝下截肢、全膝关节置换术和自发性脑室内出血后的脑室造口术。没有大出血、血栓事件或死亡,也没有出现新的抑制剂。我们的实际经验表明,在emicizumab预防下,PwHA患者可以安全地进行小手术和大手术。在不同的侵入性手术和复杂的临床情况下,需要更多的数据来优化其他止血药物的剂量/持续时间。
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Management and Outcomes of Invasive Procedures in Individuals with Hemophilia A on Emicizumab Prophylaxis: A Single Center Experience
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.
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