St. Jude Children's Research Hospital, Memphis, USA.
IRCCS Cà Granda Foundation, Maggiore Hospital, Milan, Italy.
Haemophilia. 2017 Nov;23(6):821-831. doi: 10.1111/hae.13279. Epub 2017 Jul 27.
While there is substantial literature addressing the principles of general management of haemophilia, literature on perioperative management of haemostasis is scarce.
The aim of this study was to better understand perioperative management among congenital haemophilia B patients (without inhibitors) and to gain insights into real-world surgical practices.
A systematic literature review, with an emphasis on haemophilia B, was conducted using EMBASE , Medline and the Cochrane Library. Studies from 1974 to June 2015 were accessed, and 132 studies were eligible for the full-study review. An international expert panel with five haematologists and one surgeon reviewed the resulting literature and provided further insights.
The literature review revealed that documented experience in the perioperative management of bleeding risk in haemophilia B patients is relatively scarce. Therefore, the review was amended to provide a comprehensive overview of the perioperative management for haemophilia A and B patients; the expert panel applied a particular focus to haemophilia B. Several gaps were identified in the literature including the lack of consensus on defining surgery in terms of bleeding risk, optimal factor levels during surgery and lack of robust evidence on surgical outcomes. The ensuing discussions with the expert panel provided validation of some of the results from the systematic literature review and proposed future directions for perioperative management. Suggestions included collaboration with haemophilia treatment centres (HTCs) to collect real-world data on perioperative management, establishing the need for optimal factor level monitoring practice, and the appropriate adoption of extended half-life products in clinical settings.
虽然有大量文献探讨了血友病的一般管理原则，但关于止血围手术期管理的文献却很少。
本研究旨在更好地了解先天性乙型血友病（无抑制剂）患者的围手术期管理，并深入了解实际的手术实践。
采用 EMBASE、Medline 和 Cochrane Library 进行了系统的文献回顾，重点关注乙型血友病。检索了 1974 年至 2015 年 6 月的文献，有 132 项研究符合全文审查标准。一个由五名血液学家和一名外科医生组成的国际专家小组对相关文献进行了回顾，并提供了进一步的见解。
文献回顾表明，关于乙型血友病患者围手术期出血风险管理的记录经验相对较少。因此，该综述进行了修订，以全面概述血友病 A 和 B 患者的围手术期管理；专家组特别关注乙型血友病。文献中存在一些空白，包括在手术出血风险、手术期间最佳因子水平以及缺乏关于手术结果的有力证据等方面缺乏共识。随后与专家组的讨论验证了系统文献综述的一些结果，并提出了围手术期管理的未来方向。建议包括与血友病治疗中心（HTC）合作收集围手术期管理的真实数据，建立最佳因子水平监测实践的必要性，以及在临床环境中适当采用长半衰期产品。
