镰状细胞-β+-地中海贫血：利比里亚的血液学和临床研究。
Sickle cell-beta +-thalassaemia: a haematological and clinical study in Liberia.
作者信息
Bienzle U, Kappes R, Reimer A, Feldheim M, Tischendorf F W, Kohne E
出版信息
Blut. 1983 Nov;47(5):279-85. doi: 10.1007/BF00319897.
PMID:6194836
Abstract
Clinical and haematological features of 20 patients of several Liberian ethnic groups with sickle cell-beta +-thalassaemia are reported. Haemoglobin analysis showed increased Hb A2 values, high Hb A levels (median 25%), variable amounts of Hb F and a slight imbalance of non alpha/alpha globin chain synthesis ratios. The clinical and other haematological findings varied but the disease seems to run a relatively mild course in the majority of the patients.
摘要
报告了20例来自利比里亚多个种族群体的镰状细胞-β+-地中海贫血患者的临床和血液学特征。血红蛋白分析显示Hb A2值升高、Hb A水平较高（中位数为25%）、Hb F含量可变以及非α/α珠蛋白链合成比率略有失衡。临床和其他血液学检查结果各不相同，但该病在大多数患者中似乎病程相对较轻。
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