Department of Haematology, Oslo University Hospital, Oslo, Norway.
Research Institute of Internal Medicine, Oslo University Hospital, Oslo, Norway.
Haemophilia. 2024 Jan;30(1):98-105. doi: 10.1111/hae.14899. Epub 2023 Nov 20.
The impact of moderate haemophilia on health-related quality of life (HRQoL) and physical activity (PA) is not well known. In previous studies, persons with factor VIII/factor IX activity (FVIII/FIX:C) below 3 IU/dL were associated with a more severe bleeding phenotype than predicted.
To explore HRQoL and PA in patients with moderate haemophilia A (MHA) and B (MHB).
A cross-sectional, multicentre study covering patients with MHA and MHB in Sweden, Finland, and Norway. HRQoL was assessed with the EuroQoL 5-Dimensions (EQ-5D) form and PA with the International Physical Activity Questionnaire among participants aged ≥15 years.
We report on 104 patients aged 15-84 years from the MoHem study. Overall, EQ-5D utility was .85 (median) (Q1-Q3 0.73-1.0) with corresponding visual analogue scale (VAS) 80 (70-90), which were similar regardless of treatment modality, FVIII/FIX:C, and MHA or MHB. Pain and mobility were most frequently affected dimensions. Utility (r = -.54), VAS (r = -.42), and PA (r = -.32) correlated negatively with arthropathy (HJHS). Only patients aged 41-50 years displayed lower utility (p = .02) and VAS (p < .01) than the Norwegian population norm. Patients on prophylaxis aged 35-54 years reported higher PA than those treated on-demand (p = .01).
Haemophilic arthropathy had negative impact on HRQoL and PA in Nordic patients with moderate haemophilia. Middle-aged patients captured lower utility and VAS than observed in the general population. Tailored prophylaxis and improved joint health may influence positively on HRQoL and PA also in moderate haemophilia.
中度血友病对健康相关生活质量（HRQoL）和身体活动（PA）的影响尚不清楚。在以前的研究中，因子 VIII/因子 IX 活性（FVIII/FIX：C）低于 3 IU/dL 的患者与预测相比具有更严重的出血表型。
探讨瑞典、芬兰和挪威中度血友病 A（MHA）和 B（MHB）患者的 HRQoL 和 PA。
一项横断面、多中心研究，涵盖了瑞典、芬兰和挪威的 MHA 和 MHB 患者。年龄≥15 岁的参与者使用欧洲五维健康量表（EQ-5D）和国际体力活动问卷评估 HRQoL 和 PA。
我们报告了来自 MoHem 研究的 104 名年龄在 15-84 岁的患者。总体而言，EQ-5D 效用为.85（中位数）（Q1-Q3 0.73-1.0），相应的视觉模拟量表（VAS）为 80（70-90），无论治疗方式、FVIII/FIX：C 以及 MHA 或 MHB 如何，这些结果均相似。疼痛和活动能力是受影响最频繁的维度。效用（r = -.54）、VAS（r = -.42）和 PA（r = -.32）与血友病性关节炎（HJHS）呈负相关。只有 41-50 岁的患者的效用（p =.02）和 VAS（p <.01）低于挪威人群的正常值。35-54 岁接受预防治疗的患者比按需治疗的患者报告的 PA 更高（p =.01）。
血友病性关节炎对北欧中度血友病患者的 HRQoL 和 PA 产生负面影响。中年患者的效用和 VAS 低于一般人群。量身定制的预防治疗和改善关节健康状况可能对中度血友病患者的 HRQoL 和 PA 产生积极影响。
