Li Changgang, Zhang Xinsheng, Zhao Yongqiang, Wu Runhui, Hu Qun, Xu Weiqun, Sun Jing, Yang Renchi, Li Xiaojing, Zhou Rongfu, Lian Shinmei, Gu Jian, Wu Junde, Hou Qingsong
a Department of Hematology and Oncology , Shenzhen Children's Hospital , Shenzhen , China.
b Hemophilia Treatment Centre, Shandong Blood Centre , China.
Curr Med Res Opin. 2017 Jul;33(7):1223-1230. doi: 10.1080/03007995.2017.1310720. Epub 2017 Apr 25.
The first recombinant factor VIII (rFVIII) product was launched in China in 2007. However, until now, no study has been conducted to describe the efficacy and safety of prophylaxis with rFVIII in Chinese pediatric patients with hemophilia A (HA).
To summarize the efficacy and safety data on prophylaxis with rFVIII in Chinese pediatric patients with HA.
ReCARE (Retrospective study in Chinese pediatric hemophilia A patients with rFVIII contained regular prophylaxis) was a retrospective study conducted in 12 hemophilia treatment centers (HTCs) across China. The primary endpoints included reduction in annualized bleeding rate (ABR); the secondary endpoints included evaluation of joint function (number and sites of target joints) using Gilbert score and Hemophilia Joint Health Score (HJHS), quality of life (QoL) and factors affecting treatment choices. Safety assessment of rFVIII was also conducted.
We analyzed a total of 183 male pediatric patients (mean age, 7.1 ± 4.23 years) who received prophylaxis between 1 November 2007 and 31 May 2013. Compared with baseline, prophylaxis with rFVIII significantly reduced overall annualized joint bleed rate (AJBR) (p < .001) and ABR (p < .001). Inhibitor formation was reported in 5 (2.7%) patients and hemarthrosis was reported in 1 patient. The mean number of target joints was positively related to age (p < .001) and weight (p = .003) at baseline. Responses from survey questionnaires reported that effective bleeding control, joint protection, improvement in quality of life, favorable medical insurance policies, and economic capability were reasons for choosing prophylaxis.
Prophylaxis with rFVIII reduced bleeding and number of target joints, even with a low-dose regimen, in Chinese pediatric patients with HA. Other than the efficacy and safety, factors such as poor disease control, improved economic stability and stable financial support made prophylaxis as an attractive treatment option. ClinicalTrials.gov ID: NCT02263066.
首个重组凝血因子VIII(rFVIII)产品于2007年在中国上市。然而,截至目前,尚未有研究描述rFVIII在中国儿童甲型血友病(HA)患者中进行预防治疗的疗效和安全性。
总结rFVIII在中国儿童HA患者中进行预防治疗的疗效和安全性数据。
ReCARE(对接受rFVIII常规预防治疗的中国儿童甲型血友病患者的回顾性研究)是一项在中国12个血友病治疗中心开展的回顾性研究。主要终点包括年化出血率(ABR)的降低;次要终点包括使用吉尔伯特评分和血友病关节健康评分(HJHS)评估关节功能(目标关节的数量和部位)、生活质量(QoL)以及影响治疗选择的因素。同时也对rFVIII进行了安全性评估。
我们分析了2007年11月1日至2013年5月31日期间接受预防治疗的183例男性儿童患者(平均年龄7.1±4.23岁)。与基线相比,rFVIII预防治疗显著降低了总体年化关节出血率(AJBR)(p<0.001)和ABR(p<0.001)。有5例(2.7%)患者报告出现抑制剂,1例患者报告出现关节积血。基线时目标关节的平均数量与年龄(p<0.001)和体重(p=0.003)呈正相关。调查问卷的反馈报告称,有效的出血控制、关节保护、生活质量改善、良好的医疗保险政策和经济能力是选择预防治疗的原因。
在中国儿童HA患者中,即使采用低剂量方案,rFVIII预防治疗也能减少出血和目标关节数量。除了疗效和安全性外,疾病控制不佳、经济稳定性改善和稳定的经济支持等因素使预防治疗成为一种有吸引力的治疗选择。ClinicalTrials.gov标识符:NCT02263066。