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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