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Delta beta-thalassemia in a Mexican family: clinical differences among homozygotes.
Hemoglobin ; 2(6): 513-29, 1978.
Article in En | MEDLINE | ID: mdl-750552
Three delta beta-thalassemia homozygotes were found in a Mexican family. Both parents and two sibling had heterozygous delta beta-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 Ggamma and Agamma chains as in BgammaAgamma-delta beta-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 severe in vitro chain synthesis imbalance than her homozygous sisters. Comparison of these cases with other GgammaAgamma-delta beta-thalassemia homozygotes and with GgammaAgamma-HPFH homozygotes indicates the possibility that the proliferation of F-cell precursors may be defective in delta beta-thalassemia.
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Collection: 01-internacional Database: MEDLINE Main subject: Thalassemia / Homozygote Limits: Child / Female / Humans Country/Region as subject: Mexico Language: En Journal: Hemoglobin Year: 1978 Document type: Article Country of publication: United kingdom
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Collection: 01-internacional Database: MEDLINE Main subject: Thalassemia / Homozygote Limits: Child / Female / Humans Country/Region as subject: Mexico Language: En Journal: Hemoglobin Year: 1978 Document type: Article Country of publication: United kingdom