益髓生血颗粒治疗血红蛋白H病25例临床观察

Clinical observation of Yisui Shengxue Granule in treating 25 patients with hemoglobin H disease

目的:研究益髓生血颗粒治疗血红蛋白H(hemoglobin H,HbH)病的临床效果及安全性。方法:观察益髓生血颗粒治疗前后25例HbH病患者血红蛋白(hemoglobin,Hb)浓度、红细胞(red bloodcell,RBC)计数、HbH含量及网织红细胞(reticulocyte,Ret)计数的动态变化及疗效;同时检测患者的基因类型,比较益髓生血颗粒治疗前后不同基因型患者血液参数的变化及疗效。结果:25例患者自服药第1个月起至疗程(3个月)结束,血液参数Hb、RBC、Ret水平均明显提高,与治疗前比较,差异有统计学意义(P<0.01)。经益髓生血颗粒治疗后,12例基因缺失型患者Hb、RBC、Ret明显提高(P<0.05,P<0.01),13例基因非缺失型患者Hb、Ret明显提高(P<0.05,P<0.01)。25例患者经3个月治疗后的总有效率为84%,中药对基因缺失型和非缺失型患者的疗效比较,差异无统计学意义。治疗期间患者未出现任何不良反应。结论:益髓生血颗粒治疗HbH病疗效显著,用药安全,可明显提高患者Hb、RBC、Ret水平,尤其以基因缺失型患者为佳。

Objective：To investigate the clinical efficacy and safety of Yisui Shengxue Granule（YSSXG）,a compound traditional Chinese herbal medicine for reinforcing kidney and nourishing blood,in treating hemoglobin H（HbH）disease.Methods：YSSXG was given orally to 25 patients with HbH disease in Guangxi Zhuang Autonomous Region（high incidence area for HbH disease in China）for 3 months as one therapeutic course,3 times a day,10 g YSSXG was given each time（dose of YSSXG for children should be reduced properly）,and blood transfusion was not given to HbH patients during the course of treatment.The levels of hemoglobin（Hb）,red blood cell（RBC）,HbH and reticulocyte（Ret）were observed before and after YSSXG treatment,and side effects were observed during the course of treatment.Meanwhile,the genotype was examined,and the clinical efficacy of YSSXG in treating HbH patients with different genotype was evaluated.Results：The levels of Hb,RBC and Ret were obviously increased after YSSXG treatment from the first month to the end of treatment（P〈0.01）.After YSSXG treatment,the levels of Hb,RBC,Ret in 12 HbH patients with gene deletion were elevated（P〈0.05,P〈0.01）,and the levels of Hb and Ret in 13 HbH patients with gene non-deletion were increased obviously（P〈0.05,P〈0.01）.The total response rate was 84% after 3-month treatment,and there was no statistical difference in clinical efficacy between gene deletion HbH patients and non-deletion HbH patients.No adverse effect was observed during the course of treatment.Conclusion：YSSXG is effective and safe for treatment of HbH disease.YSSXG can improve the levels of Hb,RBC and Ret in HbH patients,especially in gene deletion HbH patients.