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α地中海贫血和β地中海贫血特征:基于红细胞指数和锌原卟啉的生物学差异

Alpha-thalassaemia and beta-thalassaemia traits: biological difference based on red cell indices and zinc protoporphyrin.

作者信息

Han P, Fung K P, Teo C P, Tam L P

机构信息

Department of Laboratory Medicine, National University Hospital, Singapore.

出版信息

Clin Lab Haematol. 1990;12(2):169-76.

PMID:2208947
Abstract

alpha-thalassaemia and beta-thalassaemia traits are two commonly encountered haemoglobinopathies in South East Asia. Both present with hypochromia and microcytosis. The use of modern electronic or optical cell counters which measure accurately the red cell parameters has allowed for an initial quick screening for the presence of thalassaemia. Seven red cell parameters were measured by the Technicon H1 cell analyser--red cell count, Hb, MCV, MCH, red cell distribution width (RDW) and haemoglobin distribution width (HDW). Discriminant analysis of these parameters and zinc protoprophyin (ZP) indicates that in healthy individuals, alpha-thalassaemia can be differentiated from beta-thalassaemia with an accuracy of 86% when analysed as a group. In hospitalized patients the accuracy dropped to 71% due to biases of concomitant illness. The two most important parameters for indicating differences between alpha- and beta-thalassaemia are MCV and ZP. The results confirm further that though alpha- and beta-thalassaemia have the same phenotypic expression they differ in their biology.

摘要

α地中海贫血和β地中海贫血特征是东南亚地区两种常见的血红蛋白病。两者均表现为低色素性和小红细胞症。使用能够精确测量红细胞参数的现代电子或光学血细胞计数器,可对地中海贫血的存在进行初步快速筛查。通过Technicon H1细胞分析仪测量了七个红细胞参数——红细胞计数、血红蛋白、平均红细胞体积(MCV)、平均红细胞血红蛋白含量(MCH)、红细胞分布宽度(RDW)和血红蛋白分布宽度(HDW)。对这些参数和锌原卟啉(ZP)进行判别分析表明,在健康个体中,将α地中海贫血作为一组进行分析时,与β地中海贫血的鉴别准确率可达86%。在住院患者中,由于合并疾病的偏差,准确率降至71%。用于指示α地中海贫血和β地中海贫血之间差异的两个最重要参数是MCV和ZP。结果进一步证实,尽管α地中海贫血和β地中海贫血具有相同的表型表达,但它们在生物学上存在差异。

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