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δβ-Thalassemia in a Mexican Family: Clinical Differences Among Homozygotes

 

作者: ReyesGuillermo Ruiz,   PiñaArturo,   FeliceA. E.,   GravelyM. E.,   HuismanT. H. J.,  

 

期刊: Hemoglobin  (Taylor Available online 1978)
卷期: Volume 2, issue 6  

页码: 513-529

 

ISSN:0363-0269

 

年代: 1978

 

DOI:10.3109/03630267809005353

 

出版商: Taylor&Francis

 

数据来源: Taylor

 

摘要:

Three→β-thalassemia homozygotes were found in a Mexican family. Both parents and two siblings had heterozygous 63-thalassemia with about 10% Hb F, mild microcytosis and mild hypochromia, while three siblings were normal. Hb F, which was the only Hb component in the homozygotes, had equal quantities of Gγand Aγchains as in GγAγ-→β-thalassemia. The homozygotes had comparable erythrocytic indices which were about the same as those of the heterozygotes. However, two were clinically and hematologically healthy but the third had a severe chronic hemolytic anemia and a more severein vitrochain synthesis imbalance than her homozygous sisters. Comparison of these cases with other GγAγ63-thalassemia homozygotes and with GγAγHPFH homozygotes indicates the possibility that the proliferation of F-cell precursors may be defective in→β-thalassemia.

 

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