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HbOThrace特征、HbOThrace血红蛋白病及HbOThrace/Hbβ0血红蛋白病:118例回顾性研究

HbOThrace trait, HbOThrace hemoglobinopathy and HbOThrace/Hb beta zero hemoglobinopathy: a retrospective study of 118 cases.

作者信息

Vassiliadou D, Papadopoulos V, Konstantinidou C, Arnaoutoglou M

机构信息

Department of Hematology, Xanthi General Hospital, Greece.

出版信息

Folia Med (Plovdiv). 1998;40(3B Suppl 3):46-50.

PMID:10205993
Abstract

HbOThrace is an abnormal hemoglobin which is constructed by defective beta chains (Glu121-->Lys121). It mostly characterises the Muslim minority of the Thrace region. During the last 6 years, our department detected HbOThrace in 118 cases. Four groups were formed, the first consisted of cases of HbOThrace trait with normal iron and ferritin levels, the second of cases of HbOThrace hemoglobinopathy, the third of cases of HbOThrace/Hb beta zero hemoglobinopathy and the fourth of cases of HbOThrace trait with low iron and ferritin levels. The second, third and fourth group were compared with the first one. The conclusion is that the presence of HbOThrace leads to a mild hypochromic anemia, with no clinical findings, but the coexistence of beta zero gene or iron deficiency produces more pronounced laboratory findings and even clinically evident anemia.

摘要

HbO色雷斯是一种异常血红蛋白,由缺陷的β链(谷氨酸121→赖氨酸121)构成。它主要在色雷斯地区的穆斯林少数群体中出现。在过去6年中,我们科室检测到118例HbO色雷斯病例。分为四组,第一组是铁和铁蛋白水平正常的HbO色雷斯特征病例,第二组是HbO色雷斯血红蛋白病病例,第三组是HbO色雷斯/Hbβ零地中海贫血病例,第四组是铁和铁蛋白水平低的HbO色雷斯特征病例。将第二、第三和第四组与第一组进行比较。结论是,HbO色雷斯的存在会导致轻度低色素性贫血,无临床症状,但β零基因或缺铁的共存会产生更明显的实验室检查结果,甚至出现临床明显的贫血。

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