地中海贫血(简称地贫)是一组以珠蛋白链合成受到部分或完全抑制为特点的遗传性慢性溶血性疾病。目前对地贫的研究多集中于探讨其分子病理学,以及某些临床表现的发病机理。重型β地贫红细胞(RBC)的破坏率是正常RBC的2～5倍,~(51)Cr标记细胞的半数消除率是正常的7～10倍。尽管地贫病人骨髓红系增殖活跃,但RBC从骨髓到外周血的最大释放 Thalassemia (thalassemia) is a group of hereditary chronic haemolytic diseases characterized by partial or complete inhibition of globin chain synthesis. At present, the research on thalassemia mostly focuses on its molecular pathology and the pathogenesis of some clinical manifestations. The destruction rate of heavy β-thalassemia erythrocytes (RBCs) is 2 to 5 times that of normal RBCs, and the half-elimination rate of ~ (51) Cr-labeled cells is 7 to 10 times normal. Although erythroblasts proliferate actively in patients with thalassemia, the maximum release of RBC from bone marrow to peripheral blood