Hospital Universitario La Paz, Madrid, Spain.
Hospital Universitario y Politécnico La Fe, Valencia, Spain.
Haemophilia. 2024 Mar;30(2):513-522. doi: 10.1111/hae.14924. Epub 2024 Jan 28.
Joint damage due to haemarthrosis can be effectively monitored with point-of care ultrasound using the Haemophilia Early Arthropathy Detection with US (HEAD-US) scoring system. A post hoc comparative analysis of the joint status of patients with severe haemophilia A (HA) or B (HB) was performed.
The databases of two observational, cross-sectional studies that recruited patients with HA or HB from 12 Spanish centres were analysed to compare the status of the elbows, knees and ankles in patients with severe disease according to treatment modality. The HEAD-US score was calculated in both studies by the same trained operators.
Overall, 95 HA and 41 HB severe patients were included, with a mean age of 35.2 ± 11.8 and 32.7 ± 14.2 years, respectively. The percentage of patients who received prophylaxis, over on-demand (OD) treatment, was much higher in HA (91.6%) than in HB (65.8%) patients. With a similar number of target joints, the HEAD-US score was zero in 6.3% HA and 22.0% HB patients (p < .01), respectively. The HA population showed significantly worse HEAD-US scores. Whilst osteochondral damage occurred more frequently in patients OD or tertiary prophylaxis, our data suggest that articular damage is less prominent in primary/secondary prophylaxis, regardless of the type of haemophilia. These latter treatment modalities were also associated with a lower prevalence of synovial hypertrophy, particularly in HB patients.
This post hoc analysis indicates that joint status seems to be significantly influenced by haemophilia type (HA or HB) and treatment modality in these severe Spanish populations with severe disease. Continuing HEAD-US monitoring for the early detection and management of intra-articular abnormalities, as well as more efficiently tailored therapies should be warranted.
通过使用即时超声检测血友病性关节炎(Haemophilia Early Arthropathy Detection with US,HEAD-US)评分系统,关节内积血导致的关节损伤可得到有效监测。本研究对严重甲型血友病(haemophilia A,HA)或乙型血友病(haemophilia B,HB)患者的关节状况进行了事后比较分析。
对西班牙 12 个中心招募的 HA 或 HB 患者进行的两项观察性、横断面研究的数据库进行了分析,以比较根据治疗方式严重疾病患者肘部、膝部和踝关节的状况。在两项研究中,由同一名经过培训的操作人员计算 HEAD-US 评分。
共纳入 95 例 HA 和 41 例 HB 严重患者,平均年龄分别为 35.2±11.8 岁和 32.7±14.2 岁。与按需治疗(on-demand,OD)相比,接受预防治疗(prophylaxis)的 HA 患者(91.6%)比例远高于 HB 患者(65.8%)。在具有相似数量的靶关节中,HA 患者的 HEAD-US 评分为 0 的比例为 6.3%,而 HB 患者为 22.0%(p<0.01)。HA 患者的 HEAD-US 评分明显更差。尽管在 OD 或三级预防患者中更常发生骨软骨损伤,但我们的数据表明,无论血友病类型如何,关节内损伤在初级/二级预防中并不明显。这些治疗方式还与滑膜增生的发生率较低相关,尤其是在 HB 患者中。
本事后分析表明,在这些严重疾病的西班牙人群中,关节状况似乎明显受血友病类型(HA 或 HB)和治疗方式的影响。应继续进行 HEAD-US 监测,以早期发现和管理关节内异常,并制定更有效的靶向治疗方案。