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原发性骨髓纤维化中的平衡珠蛋白合成

Balanced globin synthesis in idiopathic myelofibrosis.

作者信息

Ballas S K, Krasnow S H

出版信息

Clin Chim Acta. 1981 Mar 5;110(2-3):255-9. doi: 10.1016/0009-8981(81)90354-5.

Abstract

Four patients with idiopathic myelofibrosis were found to have microcytic and/or hypochromic red cell indexes. The alpha/beta globin synthetic ratio determined by incubating peripheral blood with [14C]leucine was within normal limits in all patients studied. This is unlike a recent report of acquired hemoglobin H disease with decreased alpha/beta synthetic ratio in primary myelofibrosis. This indicates that mechanisms other than alpha-thalassemia-like defects may also be involved in the production of microcytic and hypochromic red cells in myelofibrosis.

摘要

4例特发性骨髓纤维化患者被发现存在小红细胞和/或低色素性红细胞指数。通过用[14C]亮氨酸孵育外周血测定的α/β珠蛋白合成比率在所有研究患者中均在正常范围内。这与最近一篇关于原发性骨髓纤维化中获得性血红蛋白H病且α/β合成比率降低的报道不同。这表明除了α地中海贫血样缺陷之外的机制也可能参与骨髓纤维化中小红细胞和低色素性红细胞的产生。

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