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

1978; Taylor & Francis; Volume: 2; Issue: 6 Linguagem: Inglês

10.3109/03630267809005353

1532-432X

Guillermo Ruíz Reyes, Arturo Piña-Camara, Alex E. Felice, M. E. Gravely, T. H. J. Huisman,

Neonatal Health and Biochemistry

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.