Winichagoon Pranee, Svasti Saovaros, Munkongdee Thongperm, Chaiya Wantana, Boonmongkol Piatip, Chantrakul Nawarath, Fucharoen Suthat
Thalassemia Research Center, Institute of Science and Technology for Research and Development, the Department of Biochemistry, the Department of Biochemistry, Siriraj Hospital, Mahidol University, Bangkok, Thailand.
Transl Res. 2008 Oct;152(4):178-84. doi: 10.1016/j.trsl.2008.08.004. Epub 2008 Oct 1.
Basic diagnosis of hemoglobinopathies can be performed by analysis of erythrocyte indices as well as by the separation and quantification of the common hemoglobin (Hb) fractions Hb A(2), Hb S, Hb C, Hb D, Hb E, and Hb F. This study used an automatic capillary zone electrophoresis system to diagnose various types of hemoglobinopathies common in the Thai population. A total of 459 adults were recruited, which consisted of normal, various types of thalassemia carriers, and thalassemia patients with different genotypes. Hb types and quantification of all Hb components were determined by an automated capillary zone electrophoresis. The automatic capillary electrophoresis system can separate and quantitate Hbs A, F, E, A(2), Constant Spring (CS), H, and Bart's in a way that is comparable with other Hb analysis methods. Moreover, the Hb A(2) peak can be distinguished clearly from the Hb E peak in individuals who carry Hb E. The slightly increased levels of Hb A(2), 3.5% +/- 0.4%, which is shown in the carriers of Hb E, confirm that Hb E is the silent phenotype of beta(+)-thalassemia.
血红蛋白病的基本诊断可通过分析红细胞指数以及分离和定量常见血红蛋白(Hb)组分Hb A(2)、Hb S、Hb C、Hb D、Hb E和Hb F来进行。本研究使用自动毛细管区带电泳系统诊断泰国人群中常见的各种类型血红蛋白病。共招募了459名成年人,包括正常人群、各种类型的地中海贫血携带者以及不同基因型的地中海贫血患者。通过自动毛细管区带电泳测定Hb类型和所有Hb组分的定量。自动毛细管电泳系统能够以与其他Hb分析方法相当的方式分离和定量Hbs A、F、E、A(2)、恒春型(CS)、H和Bart's。此外,在携带Hb E的个体中,Hb A(2)峰可与Hb E峰清晰区分。Hb E携带者中显示Hb A(2)水平略有升高,为3.5%±0.4%,证实Hb E是β(+)-地中海贫血的静止型。