Blood · 1964 · 114 citations · 27 references
MedicineGeneticsHematologyHuman GeneticsMedical GeneticsPediatric HematologyAnemiaCongenital DisordersHematologic DisordersHemoglobin PhysiologyHemolytic DisordersRed Blood Cell DisordersAlpha Thalassemia Prenatal DiagnosisAlpha-thalassemia In NewbornsAnemia Screening In PregnancyBeta-thalassemia In NewbornsHemoglobinopathy ScreeningPediatric HemoglobinopathiesPregnancy-related Anemia
Abstract The features of hereditary persistence of fetal hemoglobin in six Greek families are described. In eight cases this abnormality was associated with typical β-thalassemia; in all these cases Hb A was the major hemoglobin component. The clinical and hematologic picture was that of mild thalassemia. The findings are compared to relevant observations in cases of Negro origin and several differences are stressed. It is tentatively concluded that the inherited abnormality occurring in these families is responsible for an incomplete shift from the fetal to the adult type of hemoglobinopoiesis, i.e., from synthesis of γ-chains to the production of β-and δ-chains.
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