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β-Thalassemia intermedia Homozygous for Normal Hemoglobin A2β-Thalassemia

 

作者: Muzaffer Aksoy,   Engin Bermek,   Gönül Almiş,   Abdullah Kutlar,  

 

期刊: Acta Haematologica  (Karger Available online 1982)
卷期: Volume 67, issue 1  

页码: 57-61

 

ISSN:0001-5792

 

年代: 1982

 

DOI:10.1159/000207025

 

出版商: S. Karger AG

 

关键词: β-Thalassemia intermedia;Globin chain synthesis;Low HbF inβ-thalassemia;Normal A2b-thalassemia

 

数据来源: Karger

 

摘要:

Four homozygotes for β-thalassemia with normal hemoglobins A2 and F were studied. The absence or scarcity of transfusion requirement and comparatively low hemoglobin F content were the most important findings. Both parents of 3 patients showed the findings of β -thalassemia with normal hemoglobins A2 and F. Biosynthetic studies in 2 patients and their both parents showed moderate or mild β -chain deficiency. The possible reason for this comparatively mild course of a β -thalassemia syndrome lies in a mild deficit in β -chain produc

 

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