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[应用粘弹性衍射法(激光衍射红细胞变形性测定法)对遗传性溶血性贫血红细胞变形性的研究]

[Study of erythrocyte deformability using visco-diffractometry (ektacytometry) in hereditary hemolytic anemias].

作者信息

Herrera A, Feo C J

出版信息

Nouv Rev Fr Hematol (1978). 1984;26(3):169-77.

PMID:6739286
Abstract

Deformability is a basic characteristic of red blood cells; its regulating factors are presently well known (surface volume ratio, internal viscosity, viscoelastic properties of the membrane). The contribution of each of these factors in the pathogeny of hemolytic anemias has not been fully established. We report 64 hereditary hemolytic anemias in which we studied the deformability, using a visco-diffractometric method (ektacytometer) on whole blood and various subpopulations separated by density. Comparison of the response of the cells submitted to isotonic and hypotonic media allows the determination of the respective contribution of viscosity and surface volume ratio. Deformability is always decreased in red blood cell membrane diseases and in homozygous or double heterozygous hemoglobinopathies; on the other hand, deformability is normal in all the enzymopathies studied. This decrease could be due either to one regulation factor (increase of internal viscosity easily demonstrated by this method in hereditary xerocytosis and hemoglobin CC) or to several parameters: increase of internal viscosity and decrease of surface volume ratio in hereditary spherocytosis.

摘要

可变形性是红细胞的一项基本特性;其调节因子目前已为人熟知(表面积与体积比、内部黏度、细胞膜的黏弹性)。这些因素在溶血性贫血发病机制中的各自作用尚未完全明确。我们报告了64例遗传性溶血性贫血病例,在这些病例中,我们采用黏弹性衍射法(红细胞变形仪)对全血以及通过密度分离出的各种亚群进行了可变形性研究。比较细胞在等渗和低渗介质中的反应,能够确定黏度和表面积与体积比各自的作用。在红细胞膜疾病以及纯合子或双重杂合子血红蛋白病中,可变形性总是降低的;另一方面,在所研究的所有酶病中,可变形性均正常。这种降低可能是由于单一调节因子(通过该方法在遗传性口形红细胞增多症和血红蛋白CC中很容易证明内部黏度增加),也可能是由于几个参数:遗传性球形红细胞增多症中内部黏度增加和表面积与体积比降低。

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