Abstract Number: PB1038
Meeting: ISTH 2020 Congress
Theme: Hemophilia and Rare Bleeding Disorders » Hemophilia - Clinical
Background: Prior research of prophylactic treatment for hemophilia joint diseases mainly focus on preventing joint bleeding, while lack of studies on the progression of hemophilia joint deterioration under current therapeutic regimen.
Aims: To evaluate the joint progression in hemophilia patients by conventional ultrasound (US) and Hemophilia Joint Health Score 2.1 evaluation (HJHS2.1)
Methods: 27 severe hemophilia A children were enrolled . Clinical data were collected by routine practice accompanied with US examination and HJHS2.1 assessment of both ankles, elbows and knees. According to the prophylactic dose and frequency, joints were divided into low-dose and medium-dose group, while normal joints and diseased joints were grouped based on the score of ultrasound assessment. The children were followed up for one year to evaluate joint progression.
Results: 157 joints from 27 patients showed that the variation in both US and HJHS2.1 evaluation via low-dose-treatment was more prevalent than that in medium-dose- treatment (P> 0.05). Compared with normal joints, the degree of deterioration in diseased joints was significantly higher both in US detection and HJHS assessment (P < 0.05). In our study, 74 joints were found without history of hemarthrosis. Among these joints, 18.9% of joints with variation in US scoring, whereas 14.9% of joints developed during HJHS2.1 assessment.
Conclusions: Despite the degree of joint deterioration in low-dose group has a tendency to develop faster compared with medium-dose group, the low-dose prophylactic scheme can still delay the progression of joint injury in a brief period to some extent , while the long-term effect of this regimen still needs to be ascertained. In addition, our study also suggests that worsening of the joint condition may lead to exacerbation of joints. Hence, for the comprehensive evaluation of hemophilia joint diseases, it is of great importance to combine US examination with HJHS assessment.
To cite this abstract in AMA style:
Zhang Y, Xu C, Li Y, Sun J, Gao Y, Ma F, Liu H, Yi M. Prophylactic Treatment with Low or Medium Dose Factor VIII for Children with Severe Hemophilia A in China: Evaluation of Joint Progression by Conventional Ultrasound and Hemophilia Joint Health Score [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/prophylactic-treatment-with-low-or-medium-dose-factor-viii-for-children-with-severe-hemophilia-a-in-china-evaluation-of-joint-progression-by-conventional-ultrasound-and-hemophilia-joint-health-score/. Accessed September 22, 2023.« Back to ISTH 2020 Congress
ISTH Congress Abstracts - https://abstracts.isth.org/abstract/prophylactic-treatment-with-low-or-medium-dose-factor-viii-for-children-with-severe-hemophilia-a-in-china-evaluation-of-joint-progression-by-conventional-ultrasound-and-hemophilia-joint-health-score/