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.
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 HbHpatients 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 HbHpatients 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 HbHpatients with gene deletion were elevated (P<0.05, P<0.01), and the levels of Hb and Ret in 13 HbHpatients 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 HbHpatients and non-deletion HbHpatients. 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 HbHpatients, especially in gene deletion HbHpatients.