Kutlar A, Kutlar F, Wilson J B, Headlee M G, Huisman T H
Am J Hematol. 1984 Jul;17(1):39-53. doi: 10.1002/ajh.2830170106.
Additional data are presented that were obtained with a newly developed cation-exchange high-performance liquid chromatography (HPLC) method allowing the separation of numerous normal and abnormal human hemoglobin types. The method was found to be of considerable value for the diagnosis of certain hemoglobinopathies in the adult as well as in the newborn. Definitive differentiation between, AS, SS, S-beta+-thal, SD, SC, AC, C-beta+-thal, etc in cord bloods was readily accomplished even when the samples were collected on filter paper. Analyses of the hemoglobin in cells from Hb S and Hb C heterozygotes with different numbers of alpha chain genes (alpha alpha/alpha alpha; -alpha/alpha alpha; -alpha/-alpha) after separation by a Dextran density centrifugation technique failed to show significant changes in the relative quantitation of the variant hemoglobin types. Glycosylation of hemoglobin increased greatly with an increase in red cell age. Similar analyses of red cells from a young girl with Hb S trait and Hb H disease (the --/-alpha genic arrangement) gave comparable data. Some indication was obtained that her youngest cells contained some of the beta A4 and beta S4 tetrameric hemoglobins.
本文还展示了通过新开发的阳离子交换高效液相色谱（HPLC）方法获得的额外数据，该方法能够分离多种正常和异常的人类血红蛋白类型。结果发现，该方法对于诊断成人和新生儿的某些血红蛋白病具有重要价值。即使样本是采集在滤纸上的脐带血，也能轻松实现对AS、SS、S-β⁺-地中海贫血、SD、SC、AC、C-β⁺-地中海贫血等类型的明确区分。通过葡聚糖密度离心技术分离后，对具有不同数量α链基因（αα/αα；-α/αα；-α/-α）的Hb S和Hb C杂合子细胞中的血红蛋白进行分析，结果显示变异血红蛋白类型的相对定量没有显著变化。随着红细胞年龄的增加，血红蛋白的糖基化程度大幅增加。对一名患有Hb S性状和Hb H病（--/-α基因排列）的年轻女孩的红细胞进行类似分析，得到了可比的数据。有迹象表明，她最年轻的细胞中含有一些βA4和βS4四聚体血红蛋白。
