EFFECT OF ALPHA-THALASSEMIA ON THE RHEOLOGY OF HOMOZYGOUS SICKLE-CELL DISEASE
EFFECT OF ALPHA-THALASSEMIA ON THE RHEOLOGY OF HOMOZYGOUS SICKLE-CELL DISEASE
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DOI：
10.1111/j.1365-2141.1983.tb02163.x
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发表时间：
1983-01-01
影响因子：
6.5
通讯作者：
SERJEANT, GR
中科院分区：
文献类型：
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作者：
SERJEANT, BE;MASON, KP;SERJEANT, GR
在24对匹配的纯合镰状细胞病患者（有和没有纯合α-）中进行了流变学决定因素（血浆粘度、全血粘度和红细胞变形性）的研究地中海贫血2.患有共存α-地中海贫血表现出红细胞变形能力的显著增加，所述红细胞变形能力是通过5 μ m直径的孔过滤洗涤的红细胞以及在高剪切速率（230 s-1）和标准血细胞比容（0.45）下全血的粘度测量的。这种流变学优点可以解释α-β-环糊精的有益效果。地中海贫血2型对纯合子镰状细胞病血液学参数和临床事件的影响
A study of rheological determinants (plasma viscosity, whole-blood viscosity, and erythrocyte deformability) was made in 24 matched pairs of patients with homozygous sickle cell disease, with and without homozygous .alpha.-thalassemia 2. Patients with coexisting .alpha.-thalassemia showed a significant increase in erythrocyte deformability measured as filtration of washed erythrocytes through 5 .mu.m diameter pores and also as viscosity of whole blood at high shear rate (230 s-1) and standard hematocrit (0.45). This rheological advantage may explain the beneficial effect of .alpha.-thalassemia 2 on hematological parameters and clinical events in homozygous sickle cell disease.