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接受emicizumab预防治疗的 A 型血友病患者手术管理:来自美国大型综合治疗中心的真实世界经验。

Management of people with haemophilia A undergoing surgery while receiving emicizumab prophylaxis: Real-world experience from a large comprehensive treatment centre in the US.

机构信息

Indiana Hemophilia and Thrombosis Center, Indianapolis, IN, USA.

出版信息

Haemophilia. 2021 Jan;27(1):90-99. doi: 10.1111/hae.14212. Epub 2020 Nov 27.

Abstract

INTRODUCTION

Surgery is frequently required in persons with haemophilia A (PwHA). Emicizumab, a bispecific, humanized monoclonal antibody, bridges activated factor (F) IX and FX. Management of patients undergoing surgery while receiving emicizumab is of clinical interest due to paucity of data.

AIM

Review real-world experience of PwHA with/without FVIII inhibitors who required surgery while receiving emicizumab prophylaxis.

METHODS

Data regarding peri-operative management, including type of surgery, haemostatic agent use and bleeding complications, were collected for PwHA receiving emicizumab undergoing surgery between 25/10/18 and 31/12/19 at the Indiana Hemophilia and Thrombosis Center. Analyses were exploratory and descriptive.

RESULTS

Twenty minor and five major surgeries were performed in 17 and five patients, respectively. Overall, 9/20 minor surgeries were planned to occur with emicizumab as the sole haemostatic agent; of these, four required additional coagulation factor (2 due to haematomas following port removals, 1 due to oozing at port removal site, 1 due to bleeding following squamous cell carcinoma removal). Three of the 11 minor surgeries with planned additional coagulation factor resulted in non-major bleeds; all were safely managed with additional coagulation factor. All five major surgeries were planned with additional haemostatic agents; there was 1 bleed in a patient undergoing elbow synovectomy with nerve transposition, likely triggered by physical/occupational therapy. There were no major bleeds, thrombotic events or deaths.

CONCLUSIONS

Additional haemostatic agent use is safe in PwHA undergoing surgery while receiving emicizumab. Additional data are needed to determine the optimal dosing/length of treatment of additional haemostatic agents to lower bleeding risk.

摘要

介绍

患有血友病 A(PwHA)的人经常需要接受手术。依库珠单抗是一种双特异性、人源化单克隆抗体,可桥接激活的因子(F)IX 和 FX。由于数据有限,接受依库珠单抗治疗的患者在接受手术时的管理具有临床意义。

目的

回顾在接受依库珠单抗预防治疗的同时需要手术的 PwHA 有无 VIII 因子抑制剂的真实世界经验。

方法

收集印第安纳血友病和血栓形成中心在 2018 年 10 月 25 日至 2019 年 12 月 31 日期间接受依库珠单抗治疗并接受手术的 PwHA 的围手术期管理数据,包括手术类型、止血剂使用和出血并发症。分析是探索性和描述性的。

结果

17 名患者接受了 20 次小手术,5 名患者接受了 5 次大手术。总的来说,20 次小手术中有 9 次计划仅使用依库珠单抗作为唯一止血剂;其中 4 次需要额外的凝血因子(2 次是由于取出端口后血肿,1 次是由于取出端口部位渗血,1 次是由于鳞状细胞癌切除后出血)。计划额外使用凝血因子的 11 次小手术中有 3 次出现非大出血;所有患者均通过额外使用凝血因子安全管理。所有 5 次大手术均计划使用额外的止血剂;1 名患者在肘部滑膜切除术伴神经移位术中发生出血,可能是物理/职业治疗触发的。没有大出血、血栓事件或死亡。

结论

在接受依库珠单抗治疗的 PwHA 接受手术时,额外使用止血剂是安全的。需要更多的数据来确定额外使用止血剂的最佳剂量/治疗时间,以降低出血风险。

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