HEAD-US-C quantitative ultrasound assessment scale in evaluation of joint damage in patients with moderate or severe hemophilia A received on-demand versus prophylaxis replacement therapy
10.3760/cma.j.issn.0253-2727.2018.10.006
- VernacularTitle: HEAD-US-C超声评估量表对中间型/重型血友病A患者按需和预防替代治疗关节损伤评价
- Author:
Jun LI
1
;
Wei LIU
;
Xinjuan GUO
;
Xiaoling DING
;
Bingmei LYU
;
Jing XIAO
;
Qingli SUN
;
Dongshuang LI
;
Wenfeng ZHANG
;
Jinchong ZHONG
;
Changping LI
;
Renchi YANG
1
Author Information
1. Institute of Hematology & Blood Diseases Hospital, CAMS & PUMC, Tianjin 300020, China
- Publication Type:Journal Article
- Keywords:
Hemophilia A;
Replacement therapy;
Joint disease;
Ultrasonography;
Scoring system
- From:
Chinese Journal of Hematology
2018;39(10):817-821
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To explore the evaluation of joint injury by HEAD-US-C (Hemophilic Early Arthropathy Detection with UltraSound in China, HEAD-US-C) in patients with moderate or severe hemophilia A treated with prophylaxis vs on-demand.
Methods:The patients from June 2015 to July 2017 with moderate or severe hemophilia A were examined by ultrasound imaging of the elbows, knees and ankles; Meanwhile the HEAD-US-C ultrasound assessment scale and hemophilia joint health score scale 2.1 (HJHS2.1) were used to score the joint status. The correlation between the HEAD-US-C and HJHS score was performed in prophylaxis group and on-demand group patients, respectively.
Results:A total of 925 cases of joint ultrasonography were conducted in 70 patients with moderate or severe hemophilia A. Among patients with moderate hemophilia, the median (IQR) of HEAD-US-C score and HJHS score in on-demand group were significantly higher than those in the prophylaxis group[1 (0, 6) vs 0.5 (0, 3) , z=0.177, P=0.046],[2 (0, 4) vs 2 (0, 3) z=0.375, P=0.007], even though there was no significant difference of the median (IQR) number of annualized target joints bleeding episodes between on-demand and prophylaxis groups[1 (0, 7) vs 1 (0, 5) , z=1.271, P=0.137]. Unlike in moderate cases, on-demand treatment group had more annualized target joints bleeding episodes than prophylaxis group among patients with severe hemophilia[3 (0, 8) vs 2 (0, 8) , z=0.780 P=0.037]. The prophylaxis group compared favorably with on-demand therapy group in terms of HEAD-US-C score[1 (0, 6) vs 4 (0, 7) , z=2.189, P=0.008], and HJHS score[2 (0, 5) , 4 (1, 6) , z=3646, P<0.001]for the severe hemophilia patients. The positive correlation between HEAD-US-C score and HJHS score was identified (P<0.05) , whether on-demand treatment or prophylaxis groups. The correlation coefficient between HEAD-US-C score and HJHS score in on-demand treatment and prophylaxis groups were 0.739 (95% CI 0.708-0.708) , 0.865 (95% CI 0.848-0.848) respectively, and 95% CI didn’t overlap (P<0.05) , indicating that the correlation coefficient in prophylaxis group had stronger correlation than that in on-demand group.
Conclusions:Clinical effects of prophylaxis were significantly better than those of on-demand treatment in patients with moderate or se-vere haemophilia A. HEAD-US-C scoring system could effectively evaluate joints damage in hemophilia A patients treated with on-demand or prophylaxis, companied by significantly positive correlation with HJHS clinical evaluation system, and provided objective index for clinical effect assessment.
