Department of Radiology and Nuclear Medicine, Division of Imaging and Oncology, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands.
Center for Benign Haematology, Thrombosis and Haemostasis, Van Creveldkliniek, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands.
J Thromb Haemost. 2023 May;21(5):1156-1163. doi: 10.1016/j.jtha.2023.01.035. Epub 2023 Feb 8.
Previous studies suggest that subclinical bleeding occurs in persons with hemophilia.
The aim of this study was to investigate whether patients with lifelong access to prophylaxis showed signs of previous subclinical bleeding on magnetic resonance imaging (MRI) in joints without a history of joint bleeding.
This single-center cross-sectional study included persons with severe hemophilia A on prophylaxis, aged 16 to 33 years, with lifetime bleeding records available. Per participant, 1 index joint without a history of joint bleeding was evaluated with 3-Tesla MRI, including hemosiderin sensitive sequences. MRI scans were reviewed according to the International Prophylaxis Study Group (IPSG) additive MRI scale (range, 0-17/joint). Hemosiderin deposits with/without synovial hypertrophy were considered signs of previous subclinical bleeding. Additionally, physical examination was performed, followed by ultrasound examination according to the Hemophilia Early Arthropathy Detection with Ultrasound protocol.
In 43 patients with a median age of 23.5 years, 43 joints (16 elbows, 13 knees, 14 ankles) without reported bleeds were evaluated with MRI. The median IPSG MRI score was 1 (range, 0-9). Signs of previous subclinical bleeding were observed in 7 of 43 joints (16%; 95% CI, 7-30): 7 of 7 joints showed hemosiderin deposits, with concomitant synovial hypertrophy in 2 of 7 joints. MRI changes were accompanied by swelling and ultrasound-detected synovial hypertrophy in 1 ankle only. None of the other joints showed abnormalities at physical examination and ultrasound.
In this study, 16% of the joints without reported bleeds showed signs of previous subclinical bleeding, providing evidence for subclinical bleeding in people with severe hemophilia with lifelong access to prophylaxis.
先前的研究表明,患有血友病的患者会出现亚临床出血。
本研究旨在探讨终身接受预防治疗的患者在无关节出血史的关节中是否存在磁共振成像(MRI)显示先前亚临床出血的迹象。
这是一项单中心的横断面研究,纳入了接受预防治疗的严重血友病 A 患者,年龄在 16 至 33 岁之间,并且可提供终身出血记录。每例患者均评估了 1 个无关节出血史的索引关节,采用 3T MRI 检查,包括铁敏感序列。根据国际预防研究组(IPSG)附加 MRI 量表(范围为 0-17/关节)对 MRI 扫描进行评估。有/无滑膜炎增生的含铁血黄素沉积被认为是先前亚临床出血的迹象。此外,还进行了体格检查,然后根据超声检测血友病早期关节病(Hemophilia Early Arthropathy Detection with Ultrasound,HEAT)方案进行超声检查。
在 43 名中位年龄为 23.5 岁的患者中,评估了 43 个关节(16 个肘部、13 个膝关节、14 个踝关节),这些关节均无报告的出血。IPSG MRI 评分中位数为 1(范围为 0-9)。在 43 个关节中,有 7 个(16%;95%置信区间为 7-30%)观察到先前亚临床出血的迹象:7 个关节均显示含铁血黄素沉积,其中 2 个关节伴有滑膜炎增生。仅在 1 个踝关节中,MRI 改变伴有肿胀和超声检测到的滑膜炎增生。其他关节在体格检查和超声检查中均无异常。
在这项研究中,无报告出血的关节中有 16%显示出先前亚临床出血的迹象,为终身接受预防治疗的严重血友病患者存在亚临床出血提供了证据。