Gualtierotti Roberta, Solimeno Luigi Piero, Peyvandi Flora
Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy.
Res Pract Thromb Haemost. 2024 Jan 3;8(1):102314. doi: 10.1016/j.rpth.2023.102314. eCollection 2024 Jan.
The advent of novel effective treatments and the identification of the need to achieve a higher trough level for persons with hemophilia A and B have changed the landscape of management of these patients, allowing to change the target from survival and prevention of life-threatening complications to prevention of musculoskeletal complications and improvement of quality of life. Point-of-care musculoskeletal ultrasound imaging has also improved the early recognition of joint bleeding and the differential diagnosis of acute joint pain. In addition, joint ultrasound allows the evaluation of the severity of hemophilic arthropathy in terms of synovitis and cartilage and bone damage. However, a lack of standardization in the definition of ultrasound elementary lesions of hemophilic arthropathy may lead to confusion and an incorrect evaluation of the presence and progression of joint damage. Here, we propose to start a standardization and validation process for ultrasound definitions of hemophilic arthropathy that has been planned to become a project within the Factor VIII/IX Standardization Subcommittee of the International Society on Thrombosis and Haemostasis Scientific and Standardization Committee.
新型有效治疗方法的出现，以及确定对甲型和乙型血友病患者需要达到更高谷浓度，改变了这些患者的管理格局，使得目标从生存和预防危及生命的并发症转变为预防肌肉骨骼并发症和改善生活质量。即时护理肌肉骨骼超声成像也提高了对关节出血的早期识别以及对急性关节疼痛的鉴别诊断。此外，关节超声能够根据滑膜炎以及软骨和骨损伤来评估血友病性关节病的严重程度。然而，血友病性关节病超声基本病变定义缺乏标准化可能会导致混淆，并对关节损伤的存在和进展做出错误评估。在此，我们提议启动血友病性关节病超声定义的标准化和验证过程，该过程已计划成为国际血栓与止血学会科学和标准化委员会因子VIII/IX标准化小组委员会的一个项目。
