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目的:分析异常血红蛋白J-Bangkok(Hb J-Bangkok)及其合并不同类型地中海贫血(简称地贫)的血液学表型特征。方法:收集本院进行毛细管电泳法检测的72 397例样本,对筛查出Hb J-Bangkok者采用Sanger测序法进行DNA测序鉴定和红细胞参数分析,以及跨越断裂点聚合酶链反应(Gap-PCR)法和PCR结合反向点杂交法进行地贫基因分型检测。结果:共检出31例Hb J-Bangkok,均为杂合子。男性单纯Hb J-Bangkok杂合子的血红蛋白含量(Hb)、平均红细胞体积、平均红细胞血红蛋白量、Hb J-Bangkok和Hb An 2分别为(158±13.0) g/L、(90.1±2.3) fL、(31.1±2.5) pg、(51.3±0.7)%和(2.5±0.1)%;女性分别为(124±9.3) g/L、(93.3±4.9) fL、(31.1±1.6)pg、(50.9±1.1)%和(2.6±0.2)%;Hb J-Bangkok合并-αn 3.7杂合缺失分别为124 g/L、82.1 fL、26.1 pg、49.4%和2.4%;合并--n SEA杂合缺失分别为(120±14.1) g/L、(67.7±5.7) fL、(20.5±2.6) pg、(50.1±1.9)%和(2.1±0.4)%;合并β地贫分别为(134±11.3) g/L、(71.6±0.9) fL、(21.7±1.0) pg、(92.7±0.6)%和(5.5±0.8)%,无Hb A。n 结论:单纯Hb J-Bangkok杂合子血液学表型正常,合并不同类型地贫时表型存在差异,临床应结合表型来开展遗传咨询工作。“,”Objective:To analyze the hematological phenotypes of Hb J-Bangkok and concomitant thalassemia.Methods:In total 72 397 samples were screened by using capillary electrophoresis. Samples with Hb J-Bangkok were identified by DNA sequencing and analysis of red blood cell parameters. Gap-PCR and PCR-reverse dot blotting (PCR-RDB) were used for analyzing the thalassemia genes.Results:Thirty one cases of Hb J-Bangkok were identified, all of which were heterozygotes. The hematological phenotype index (Hb, mean corpuscular volume, mean corpuscular hemoglobin, Hb J-Bangkok, Hb An 2) for male carriers of Hb J-Bangkok were (158±13.0) g/L, (90.1±2.3) fL, (31.1±2.5) pg, (51.3±0.7)% and (2.5±0.1)%, those for female carriers were (124±9.3) g/L, (93.3±4.9) fL, (31.1±1.6) pg, (50.9±1.1)% and (2.6±0.2)%, those for Hb J-Bangkok and an α n 3.7 deletion were 124 g/L, 82.1 fL, 26.1 pg, 49.4% and 2.4%, those for Hb J-Bangkok and --n SEA deletion were (120±14.1) g/L, (67.7±5.7) fL, (20.5±2.6) pg, (50.1±1.9)% and (2.1±0.4)%, and those for Hb J-Bangkok and β-thalassemia-related variants were (134±11.3) g/L, (71.6±0.9) fL, (21.7±1.0) pg, (92.7±0.6)%, (5.5±0.8)%. No Hb A was found among the Hb J-Bangkok and concomitant β-thalassemia carriers.n Conclusion:Hb J-Bangkok heterozygotes have normal hematological phenotypes, though they may show different hematological characteristics when concomitant with different types of thalassemia, for which genetic counseling should be provided accordingly.