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一名β地中海贫血杂合子女性以及瑞士型异细胞遗传性胎儿血红蛋白持续存在患者,在接受霍奇金病治疗后出现了持久的胎儿血红蛋白重新激活和血红蛋白A2降低。

Lasting Hb F reactivation and Hb A2 reduction induced by the treatment of Hodgkin's disease in a woman heterozygous for beta-thalassemia and the Swiss type of the heterocellular hereditary persistence of Hb F.

作者信息

Cech P, Testa U, Dubart A, Schneider P, Bachmann F, Guerrasio A, Beuzard Y, Schmidt P M, Clément F, Rosa J

出版信息

Acta Haematol. 1982;67(4):275-84. doi: 10.1159/000207075.

DOI:10.1159/000207075
PMID:6180586
Abstract

A remarkable augmentation of Hb F and a reduction of Hb A2 were observed in a Sicilian woman during and after a course of treatment for Hodgkin's disease. An inverse correlation between the proportion of Hb F and Hb A2 was found over an 8-year period, as well as in populations of red blood cells fractionated by density gradient. She exhibited two genetic defects, the Swiss type of heterocellular hereditary persistence of fetal hemoglobin and a beta-thalassemia trait, which were confirmed by the study of the hemoglobin synthesis and by a family study. The lasting reactivation of Hb F synthesis is attributable to the interaction of several acquired and inherited factors.

摘要

在一名西西里女性接受霍奇金病治疗期间及治疗后,观察到血红蛋白F(Hb F)显著增加,血红蛋白A2(Hb A2)减少。在8年期间以及通过密度梯度分离的红细胞群体中,发现Hb F和Hb A2的比例呈负相关。她表现出两种遗传缺陷,即瑞士型胎儿血红蛋白异细胞遗传性持续存在和β地中海贫血特征,这通过血红蛋白合成研究和家族研究得到证实。Hb F合成的持续重新激活归因于多种获得性和遗传性因素的相互作用。

相似文献

1
Lasting Hb F reactivation and Hb A2 reduction induced by the treatment of Hodgkin's disease in a woman heterozygous for beta-thalassemia and the Swiss type of the heterocellular hereditary persistence of Hb F.一名β地中海贫血杂合子女性以及瑞士型异细胞遗传性胎儿血红蛋白持续存在患者,在接受霍奇金病治疗后出现了持久的胎儿血红蛋白重新激活和血红蛋白A2降低。
Acta Haematol. 1982;67(4):275-84. doi: 10.1159/000207075.
2
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beta-Thalassemia intermedia homozygous for normal hemoglobin A2 beta-thalassemia. Study in four families.中间型β地中海贫血纯合子,伴有正常血红蛋白A2β地中海贫血。对四个家族的研究。
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