Department of Haematology, Oslo University Hospital, Oslo, Norway.
Research Institute of Internal Medicine, Oslo University Hospital, Oslo, Norway.
Haemophilia. 2020 Sep;26(5):891-897. doi: 10.1111/hae.14114. Epub 2020 Jul 27.
The prevalence of arthropathy in moderate haemophilia A (MHA) and B (MHB) is not well known.
We evaluated joint health in Nordic patients in relation to their treatment modality.
A cross-sectional, multicentre study covering MHA and MHB in Sweden, Finland and Norway. Arthropathy was evaluated by ultrasound (HEAD-US) and Haemophilia Joint Health Score (HJHS).
We report on 145 patients: median age 28 years (IQR 13-52) and 61% MHA. Baseline factor VIII/factor IX activity (FVIII/FIX:C) was 2 IU/dL (median) (IQR 2-4): lower for MHB (2 IU/dL, IQR 1-2) than MHA (3 IU/dL, IQR 2-4) (P < .01). Eighty-five per cent of MHA and 73% MHB had a history of haemarthrosis (P = .07). Age at first joint bleed was lower for MHA (5 years [median], IQR 3-7) than MHB (7 years, IQR 5-12) (P = .01). Thirty-eight per cent received prophylaxis, started at median 10 years of age (IQR 4-24). Median joint bleeds and serious other bleeds during the last 12 months were both zero (IQR 0-1). Total HEAD-US captured 0/48 points (median) (IQR 0-2) and HJHS 4/120 points (IQR 1-10) with strong correlation between them (r = .72).
FVIII/FIX: C ≤ 3 IU/dL was associated with higher HJHS (P = .04). Fifteen per cent had undergone orthopaedic surgery.
The current joint health in Nordic moderate haemophilia patients was rather good, but a subgroup had severe arthropathy.
FVIII/FIX: C ≤ 3 IU/dL and MHA were associated with a more severe bleeding phenotype. We suggest primary prophylaxis to all patients with FVIII/FIX:C ≤ 3 IU/dL.
中度血友病 A（MHA）和 B（MHB）患者的关节病患病率尚不清楚。
我们评估了北欧患者的关节健康状况及其治疗方式。
这是一项在瑞典、芬兰和挪威进行的 MHA 和 MHB 的横断面、多中心研究。通过超声（HEAD-US）和血友病关节健康评分（HJHS）评估关节病。
我们报告了 145 名患者的情况：中位年龄 28 岁（IQR 13-52），61%为 MHA。基线凝血因子 VIII/凝血因子 IX 活性（FVIII/FIX:C）为 2 IU/dL（中位数）（IQR 2-4）：MHB（2 IU/dL，IQR 1-2）低于 MHA（3 IU/dL，IQR 2-4）（P<.01）。85%的 MHA 和 73%的 MHB 有过关节积血史（P=.07）。MHA 首次关节出血年龄较低（5 岁[中位数]，IQR 3-7），MHB 为 7 岁（IQR 5-12）（P=.01）。38%的患者接受了预防治疗，中位年龄 10 岁（IQR 4-24）开始预防治疗。过去 12 个月中，关节出血和其他严重出血的中位数均为零（IQR 0-1）。总 HEAD-US 评分为 0/48 分（中位数）（IQR 0-2），HJHS 评分为 4/120 分（IQR 1-10），两者相关性较强（r=.72）。
FVIII/FIX:C≤3 IU/dL 与较高的 HJHS 相关（P=.04）。15%的患者接受过矫形手术。
目前北欧中度血友病患者的关节健康状况相当良好，但有一部分患者存在严重的关节病。
FVIII/FIX:C≤3 IU/dL 和 MHA 与更严重的出血表型相关。我们建议所有 FVIII/FIX:C≤3 IU/dL 的患者接受初级预防治疗。
