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对一例严重贫血患儿进行Hb检测,醋纤薄膜碱性(PH8.5(电泳显示含有一种与HbA_2泳动速度相同的异常Hb;酸性(PH 6.5)琼脂电泳排除HbC及O;用标准HbE及脐血(HbF)为对照作酸及碱性珠蛋白解链试验出现异常βE及r链,证明它是HbE/β~+地中海贫血双重杂合子,结合临床症状及其它检测诊断为HbE复合B~+地中海贫血病例。家系调查其父为轻型β地中海贫血;其母与患儿有相同的异常Hb,为HbE特征。近年来我国对异常Hb及Hb病进行了广泛研究,我省某些地区的Hb普查表明在某些人群中的发生率较高并作了结构分析证实。本文报导使用一般实验室所能进行的手段,确诊一例HbE/β~+地中海贫血双重杂合子。
A case of severe anemia in children with Hb detection, acetate film alkaline (PH8.5 (electrophoresis showed a HbA2 electrophoresis with the same rate of abnormal Hb; acidic (PH 6.5) agarose exclusion of HbC and O; with standard HbE And umbilical cord blood (HbF) as control for abnormal acid and alkaline globin chain test showed abnormal βE and r chain, it is HbE / β ~ + thalassemia heterozygote, combined with clinical symptoms and other tests diagnosed as HbE complex B ~ + Cases of thalassemia.Familial survey of his father as beta thalassemia; its mother and children have the same abnormal Hb, HbE characteristics.In recent years, abnormal Hb and Hb disease in our country has been extensively studied in some areas in our province Of the Hb census showed that in some populations a higher incidence and confirmed by the structural analysis.This article reports the use of general laboratories can be the means to confirm a case of HbE / β ~ + thalassemia double heterozygotes.