α-thalassemia is the most common human hereditary hemochromatosis, caused by mutations of one to all four of the α-globin genes. The severity of symptoms ranges from asymptomatic to fatal disease. Hemoglobin H disease (HbH disease) is caused by mutations affecting three α-globin genes, causing an imbalance of α-globin and β-globin, and result ing in ineffective erythropoiesis and peripheral hemolysis. This in turn causes anemia, iron overload and splenomegaly, ultimately leading to a variety of serious complications. HbH disease can be divided into deletional HbH disease and non-deletional HbH disease. Hemoglobin Constant Spring (Hb CS) is the primary type of non-deletional HbH disease in China, and clinical manifestations of non-deletional HbH disease are usually more severe. They can be diagnosed by DNA analysis, and early diagnosis is particularly important for optimal management and prognosis of HbH disease. At present, the treatment for HbH disease is primarily preventative and supportive. Blood transfusion, iron chelation and splenectomy can be used when necessary, and there are experimental data showing that treatment with Chinese medicine has a beneficial effect on HbH disease.