Vayá Amparo, Iborra Jesús, Falcó Cristina, Moreno Isabel, Bolufer Pascual, Ferrando Fernando, Pérez María Luz
Department of Clinical Pathology, La Fe University Hospital, Valencia, Spain.
Clin Hemorheol Microcirc. 2003;28(2):71-8.
In major and intermediate thalassemia a decrease in erythrocyte deformability and increased erythrocyte aggregability has been described, but few studies have dealt with the question of rheological red blood cell behaviour in minor beta and deltabeta thalassemia carriers, mostly in deltabeta, because it is a less common entity. To ascertain whether there are differences in red blood cell behaviour between minor thalassemia and controls and between both types of thalassemia trait beta and deltabeta, we determined erythrocyte deformability and aggregability in 30 beta and 30 deltabeta trait carriers diagnosed both with conventional methods and globin gene analysis, and in 40 age- and sex-matched controls. Erythrocyte deformability determined by means of the Rheodyn SSD showed a statistically significant lower Elongation Index (EI) at all the shear stresses tested in both thalassemic groups compared with controls (p<0.001). Minor beta thalassemia carriers showed lower EI than deltabeta carriers (p<0.001). Erythrocyte aggregability measured with the Myrenne aggregometer was significantly lower in both thalassemic groups than in controls (p<0.001), although no significant differences could be observed between both thalassemic groups. The rheological alterations found in thalassemia carriers are in part due to microcytosis, hypochromia and the morphological changes that characterize this kind of anaemia. The less altered deformability found in deltabeta carriers, is in agreement with the fact that it deals with a more benign trait.
在重型和中间型地中海贫血中,已有红细胞变形性降低和红细胞聚集性增加的描述,但很少有研究涉及轻型β地中海贫血和δβ地中海贫血携带者(主要是δβ地中海贫血,因其较为少见)红细胞的流变学行为问题。为确定轻型地中海贫血与对照组之间以及β型和δβ型地中海贫血特征携带者之间红细胞行为是否存在差异,我们对30名经传统方法和珠蛋白基因分析确诊的β型携带者、30名δβ型携带者以及40名年龄和性别匹配的对照组进行了红细胞变形性和聚集性测定。通过Rheodyn SSD测定的红细胞变形性显示,与对照组相比,两个地中海贫血组在所有测试切变应力下的伸长指数(EI)均有统计学意义的显著降低(p<0.001)。轻型β地中海贫血携带者的EI低于δβ地中海贫血携带者(p<0.001)。用Myrenne凝集仪测量的红细胞聚集性在两个地中海贫血组均显著低于对照组(p<0.001),尽管两个地中海贫血组之间未观察到显著差异。地中海贫血携带者中发现的流变学改变部分归因于小红细胞症、低色素血症以及这类贫血的形态学变化。δβ地中海贫血携带者中变形性改变较小,这与它是一种症状较轻的特征相符。