Department of Orthopedic Surgery, La Paz University Hospital-IdiPaz, Madrid, Spain.
Osteoarticular Surgery Research, La Paz University Hospital Institute for Health Research - IdiPAZ (La Paz University Hospital - Autonomous University of Madrid), Madrid, Spain.
Expert Rev Hematol. 2021 Oct;14(10):887-896. doi: 10.1080/17474086.2021.1977118. Epub 2021 Sep 8.
Arthropathy is the characteristic injury of hemophilia, primarily occurring in the elbows, knees, and ankles. The aim of this review is a teaching approach devoted to hemophilia treaters in under-development countries.
Current major challenges include the absence of the following therapeutic tools for all people with hemophilia (PWH) worldwide: hematological prophylaxis; well-coordinated multidisciplinary teams in specialized centers; joint aspiration of acute hemarthrosis; point-of-care ultrasonography (POC-US); exercise; treatment of synovitis by means of radiosynovectomy; treatment of mild hemophilic arthropathy with analgesics, anti-inflammatory agents, and rehabilitation medicine; intra-articular injections of corticosteroids, hyaluronic acid, platelet-rich plasma, and mesenchymal stem cells; and treatment of severe hemophilic arthropathy by surgical treatment. The future scenario in under-development countries should include all the aforementioned treatment tools plus the possibility of performing total joint arthroplasty in PWH with inhibitors; concomitant use of bypassing agents with emicizumab for PWH with inhibitors undergoing orthopedic surgery; and telemedicine.
Primary hematological prophylaxis is now the gold standard for the management of hemophilia. Acute hemarthrosis needs intense hematological management and articular aspiration; the articular situation should be scrutinized by POC-US. Synovitis can be controlled by radiosynovectomy. In cases of severe articular degeneration, surgical procedures might be required.
关节病是血友病的特征性损伤,主要发生在肘部、膝盖和踝关节。本综述的目的是为欠发达国家的血友病治疗者提供一种教学方法。
目前的主要挑战包括以下治疗工具在全世界所有血友病患者(PWH)中都无法普及:血液学预防治疗;在专门中心有良好协调的多学科团队;急性关节积血的关节抽吸术;即时超声检查(POC-US);运动;通过放射性滑膜切除术治疗滑膜炎;用镇痛药、抗炎药和康复医学治疗轻度血友病性关节炎;关节内注射皮质类固醇、透明质酸、富含血小板的血浆和间充质干细胞;以及通过手术治疗严重血友病性关节炎。欠发达国家的未来情景应包括上述所有治疗工具,加上对有抑制剂的 PWH 进行全关节置换术的可能性;对正在接受矫形手术的有抑制剂的 PWH 同时使用旁路制剂和emicizumab;以及远程医疗。
初级血液学预防治疗现在是血友病管理的金标准。急性关节积血需要强化血液学管理和关节抽吸术;关节情况应通过 POC-US 进行检查。滑膜炎可以通过放射性滑膜切除术控制。在严重关节退化的情况下,可能需要手术治疗。