一、β地贫的发病机理β地中海贫血(简称β地贫)是一组以血红蛋白的β肽链合成量减少或缺失为特征的遗传性血液病。地中海沿岸国家及我国南方部分省市都为该病的高发区。成人血红蛋白HbA是由两条α与两条β珠蛋白链组成,分别由α、β两个珠蛋白基因编码。β地贫是由 First, the pathogenesis of β thalassemia β thalassemia (referred to as β thalassemia) is a group of hemoglobin β peptide synthesis or reduced loss of hereditary hereditary disease. Mediterranean coastal countries and some provinces and cities in southern China are the high incidence of the disease. Adult hemoglobin HbA is composed of two α and two β-globin chains, which are encoded by two α-and β-globin genes, respectively. β thalassemia is by