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血红蛋白病是血红蛋白合成异常的遗传性疾病,包括地中海贫血和异常血红蛋白综合征。据不完全统计,全世界血红蛋白病基因的携带者有一亿多人。现将与临床有关的一些资料,介绍如下。正常血红蛋白的结构和组成血红蛋白(简称Hb)是由珠蛋白和直接结合氧分子的血红素构成的一种色素蛋白。其珠蛋白部分由二条α链和二条非α链组成。目前已知的人类Hb及
Hemoglobinopathies are hereditary disorders of hemoglobin synthesis that include thalassemia and abnormal hemoglobin syndrome. According to incomplete statistics, there are more than 100 million carriers of hemoglobinopathies worldwide. Now with some clinical information, described below. Structure and composition of normal hemoglobin Hemoglobin (Hb) is a pigment protein composed of globin and heme that directly binds to oxygen molecules. The globin portion consists of two α-chains and two non-α-chains. Currently known human Hb and