Objective To observe the therapeutic effect and molecular mechanism of Yisui Shengxue Granules （granules to boost marrow and engender blood） on α-hemoglobin H （α-HbH） disease. Methods Totally 25 patients with α-HbH disease in Guangxi Zhuang Autonomous Region were treated with Yisui Shengxue Granules. The therapeutic effect was observed after 3 months of treatment. The levels of hemoglobin （Hb）

platelet （PLT）

reticulocyte （Ret） and HbH were detected before and after treatment. The activity and expression of stem cell factor （SCF）

interleukin 3 （IL-3） and granulocyte-macrophage colony-stimulating factor （GM-CSF） was observed before and after treatment. Results There were 24 effective patients and the symptoms such as hot flushes

night sweats

palpitations

shortness of breath and dry throat were significantly relieved after 1

2 and 3 months of treatment （P<0.05）. Comparing with after 1-month or 2-month treatment

dry throat was significantly relieved after 3-month treatment （P<0.01）. There was no significant change after treatment in pale complexion

limp aching lumbus and knees

dizziness and tinnitus （P>0.05）. The levels of Hb and HbH were significantly increased after 2 or 3 months of treatment （P<0.05 or P<0.01）. Comparing with 1 month after treatment

the levels of Hb and HbH were significantly increased after 2 or 3 months of treatment （P<0.05 or P<0.01）. There was no significant change after treatment in the levels of red blood cell （RBC） and PLT （P>0.05）. The activity and expression of SCF and GM-CSF was not increased after treatment in t （P>0.05）. The activity and expression of IL-3 was significantly decreased after treatment （P<0.05）. Conclusion Yisui Shengxue Granules is effective for α-HbH disease and its molecular mechanism maybe relevant to regulating the activity and expression of SCF

GM-CSF and IL-3.