中度血友病A患者关节健康状况：一项横断面多中心研究

IF 3.4 3区医学 Q2 HEMATOLOGY

Research and Practice in Thrombosis and Haemostasis Pub Date : 2025-03-19 DOI:10.1016/j.rpth.2025.102737

Ilenia Calcaterra , Federico Picasso , Federica Valeri , Erminia Baldacci , Mariasanta Napolitano , Cornelia Guerrino , Ezio Zanon , Cristina Santoro , Sergio Siragusa , Carlo Martinoli , Matteo Nicola Dario Di Minno

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引用次数: 0

摘要

背景：中度血友病A （mHA）患者的关节病患病率变化很大。mHA患者通常治疗不足，这可能导致关节损伤和生活质量恶化。本研究的目的是通过即时超声（PoC-US）和临床检查来评估mHA患者的关节状态。方法对连续接受按需置换治疗的mHA患者，按照HJHS方案进行关节状态临床检查。同一天，所有患者根据HEAD-US方案进行PoC-US评估。结果共纳入51例受试者。HJHS评分中位数为2.0（IQR:0-3.0）。hhs评分0-1分的23例（45.1%），2 - 3分的17例（33.3%），3分的11例（21.6%）。HEAD-US评分中位数为2.0(IQR:1-7)， HJHS与HEAD-US的相关性有统计学意义（rho=0.732, p<0.001）。骨软骨损伤占21.6%，滑膜肥厚（HS）占29.4%。在报告hhs评分为0-1分的人群中，有13.0%的人患有HS。关节水平的分析表明，最常见的受影响的关节是踝关节，无论是骨软骨损伤还是滑膜肥厚。结论我们的研究表明，在接受按需治疗的mHA患者中，关节病变的患病率是不可忽视的，PoC-US能够在这种临床环境中检测到骨软骨损伤和HS。对关节状态进行更广泛的筛查可能有助于定制治疗并改善mHA的结果。

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Joint health status in patients with moderate hemophilia A: a cross-sectional multi-center study

Background

The prevalence of arthropathy in patients with moderate hemophilia A (mHA) is highly variable. People with mHA are often under-treated, and this may lead to joint damage and worsen quality of life. The aim of the present study was to evaluate joint status in mHA by means of point-of-care ultrasound (PoC-US) and clinical examination.

Methods

Consecutive mHA patients receiving on-demand replacement treatment underwent a clinical examination of joint status according to HJHS protocol. On the same day, all patients underwent a PoC-US assessment according to the HEAD-US protocol.

Results

A total of 51 subjects were included. The median HJHS score was 2.0(IQR:0-3.0). A 0-1 HJHS score was found in 23 mHA patients (45.1%), between 2 and 3 in 17 (33.3%) and >3 in 11 (21.6%). The median HEAD-US score was 2.0(IQR:1-7) and a statistically significant correlation between HJHS and HEAD-US was found (rho=0.732, p<0.001). Osteochondral damage was found in 21.6% patients, hypertrophic synovium (HS) was found in 29.4%. Among those reporting a 0-1 HJHS score, 13.0% showed HS. The analysis at joint level showed that the most commonly affected joint was the ankle, both for osteochondral damage and for the presence of hypertrophic synovium.

Conclusion

Our study suggests that the prevalence of arthropathy changes in patients with mHA receiving on-demand treatment is not negligible and that PoC-US is able to detect osteochondral damage as well as HS in this clinical setting. A more extensive screening of the joint status could be useful to tailor treatment and improve outcome in mHA.

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