Department of Pediatric Hemostaseology, Pediatric Hemophilia Center, Dr. von Hauner's Children's Hospital, University of Munich, Munich, Germany.
Haemophilia. 2012 May;18(3):369-74. doi: 10.1111/j.1365-2516.2011.02682.x. Epub 2011 Oct 28.
Severe haemophilia is associated with recurrent joint bleeds, which can lead to haemophilic arthropathy. Subclinical joint bleeds have also been associated with joint damage detected using magnetic resonance imaging (MRI). We investigated the development of early changes in clinically asymptomatic joints using MRI in haemophilia A or B patients receiving prophylactic therapy. In this single-centre retrospective cohort study, patients with clinical evidence of joint damage in one ankle and one clinically asymptomatic ankle, in which we performed an MRI scan of both ankles in one session, were enrolled. MRI findings were graded using a 4-point scoring system (0 = normal findings and III = severe joint damage). Since 2000, 38 MRIs in 26 patients have been performed. Starting at a median age of 4 years, 23 patients received prophylaxis 2-3 times weekly. On-demand treatment was performed in three patients. Eight patients (31%) presented with an MRI score of 0, 12 (46%) had a score of I, four (15%) had a score of II, and two (8%) had a score of III in the clinically unaffected ankle. The six patients with MRI scores of II and III had started regular prophylaxis between the ages of 2 years and 15 years; none had developed an inhibitor or experienced a clinically evident bleed in the asymptomatic ankle. During our study, five of 26 patients had a worsening of MRI findings without experiencing a joint bleed. Early morphological changes in clinically asymptomatic ankles can be detected using MRI, despite adequate prophylaxis.
重度血友病与反复关节出血有关,这可能导致血友病性关节病。亚临床关节出血也与磁共振成像(MRI)检测到的关节损伤有关。我们研究了接受预防性治疗的 A 型或 B 型血友病患者无症状关节的早期变化。在这项单中心回顾性队列研究中,招募了有一个踝关节临床关节损伤和一个临床无症状踝关节的患者,我们在一次检查中对两个踝关节进行了 MRI 扫描。使用 4 分制评分系统(0 = 正常发现和 III = 严重关节损伤)对 MRI 结果进行分级。自 2000 年以来,对 26 名患者的 38 次 MRI 进行了评估。23 名患者从 4 岁中位数开始每周接受 2-3 次预防性治疗。有 3 名患者按需治疗。在临床未受影响的踝关节中,8 名患者(31%)MRI 评分为 0,12 名患者(46%)评分为 I,4 名患者(15%)评分为 II,2 名患者(8%)评分为 III。MRI 评分为 II 和 III 的 6 名患者在 2 至 15 岁之间开始定期接受预防性治疗;在无症状踝关节中,他们均未出现抑制剂或明显出血。在我们的研究期间,26 名患者中有 5 名患者尽管接受了充分的预防治疗,但 MRI 检查结果仍恶化。尽管进行了充分的预防治疗,仍可以通过 MRI 检测到无症状踝关节的早期形态变化。