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镰状细胞贫血中的红细胞密度分布。

Erythrocyte density distribution in sickle cell anemia.

作者信息

Weems H B, Lessin L S

出版信息

Acta Haematol. 1984;71(6):361-70. doi: 10.1159/000206620.

Abstract

Density distributions were determined for sickle cell erythrocytes from 27 patients with HbSS genotype using the phthalate ester microcapillary differential flotation method of Danon and Marikovsky. Mean density distribution curves showed HbSS erythrocytes to have trimodal populations with significant increases in both dense and light cell fractions when compared to 20 normal controls of HbAA genotype (p less than 0.05). Irreversibly sickled cell (ISC) and reticulocyte counts were compared with density distributions. Integrated unit areas under the dense cell population curves correlated with ISC percentages, while corresponding unit areas for light populations correlated to a lesser extent with reticulocyte percentages. Mean cell density, D50, varied widely among patients and correlated poorly with the ISC or reticulocyte percentage; however, D50 did correlate with the net change in integrated unit areas. Several patients had repeated density distributions over a 2-year time period. All HbSS patients showed increased but variable dense cell fractions which could not be definitively correlated with the clinical state of the patient. Transfusion reproducibly resulted in a lowering of the dense cell fraction. Erythrocytes from the HbSC patients showed a uniform increase in density and absence of the large dense cell fraction seen in most HbSS patients. This method provides a simple means for quantitation of the light and dense cell fraction in blood of patients with sickling disorders and displays the profile of erythrocyte density heterogeneity for the individual sickle cell patient.

摘要

采用达农和马里科夫斯基的邻苯二甲酸酯微毛细管差示浮选法,对27例HbSS基因型患者的镰状红细胞进行密度分布测定。平均密度分布曲线显示,与20例HbAA基因型正常对照相比,HbSS红细胞具有三峰分布,致密细胞和轻细胞部分均显著增加(p小于0.05)。将不可逆镰状细胞(ISC)和网织红细胞计数与密度分布进行比较。致密细胞群体曲线下的积分单位面积与ISC百分比相关,而轻细胞群体的相应单位面积与网织红细胞百分比的相关性较小。平均细胞密度D50在患者之间差异很大,与ISC或网织红细胞百分比的相关性很差;然而,D50确实与积分单位面积的净变化相关。几位患者在两年时间内进行了多次密度分布测定。所有HbSS患者的致密细胞部分均增加但存在差异,这与患者的临床状态没有明确的相关性。输血可重复性地导致致密细胞部分降低。HbSC患者的红细胞密度均匀增加,且没有大多数HbSS患者中出现的大量致密细胞部分。该方法为镰状细胞疾病患者血液中的轻细胞和致密细胞部分定量提供了一种简单方法,并展示了个体镰状红细胞患者的红细胞密度异质性特征。

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