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轻型地中海贫血:红细胞常规检测及与缺铁性贫血的鉴别

Thalassemia minor: routine erythrocyte measurements and differentiation from iron deficiency.

作者信息

Johnson C S, Tegos C, Beutler E

出版信息

Am J Clin Pathol. 1983 Jul;80(1):31-6. doi: 10.1093/ajcp/80.1.31.

Abstract

The clinical differentiation of the causes of microcytosis is difficult because of the lack of a method for the diagnosis of alpha thalassemia. A number of laboratory tests have been proposed for the differentiation of alpha thalassemia from iron deficiency, including decision functions based on the red blood cell indices generated by electronic cell counters. The accuracy of these screening methods was assessed in 93 patients with microcytosis known to be secondary to either iron deficiency or beta thalassemia minor and, prospectively, in 26 patients with microcytosis in whom globin chain synthesis ratio was used to diagnose thalassemia. The functions evaluated were: RBC volume distribution curve; osmotic fragility; erythrocyte count; discriminant function = MCV - (5 X Hgb) - RBC - 8.4; ratio of MCH/RBC; ratio of MCV/RBC; and 0.01 X MCH X (MCV)2. A simplified method of measuring anisocytosis using the RBC volume distribution curve was significantly more accurate (P less than 0.01) in distinguishing iron deficiency from thalassemia than any of the other decision functions. Analysis of red blood cell volume distribution, although not sufficiently accurate for definitive diagnosis, appears to be a useful technic in the initial screening of patients with microcytosis and in determining which additional testing should be done.

摘要

由于缺乏诊断α地中海贫血的方法,小细胞症病因的临床鉴别存在困难。已提出多项实验室检测方法用于区分α地中海贫血和缺铁性贫血,包括基于电子细胞计数器生成的红细胞指数的判别函数。在93例已知因缺铁或轻型β地中海贫血继发小细胞症的患者中,以及前瞻性地在26例使用珠蛋白链合成比率诊断地中海贫血的小细胞症患者中,评估了这些筛查方法的准确性。所评估的函数包括:红细胞体积分布曲线;渗透脆性;红细胞计数;判别函数=平均红细胞体积-(5×血红蛋白)-红细胞-8.4;平均红细胞血红蛋白含量/红细胞比率;平均红细胞体积/红细胞比率;以及0.01×平均红细胞血红蛋白含量×(平均红细胞体积)²。使用红细胞体积分布曲线测量红细胞大小不均一性的简化方法在区分缺铁性贫血和地中海贫血方面比任何其他判别函数都显著更准确(P<0.01)。红细胞体积分布分析虽然对明确诊断不够准确,但似乎是对小细胞症患者进行初步筛查以及确定应进行哪些额外检测的有用技术。

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