This report is concerned with the evaluation of hematological parameters and of both relative (%) and absolute (mean pg/cell) quantities of the abnormal Hemoglobin (Hb) Hasharon in 53 heterozygous carriers and 7 double heterozygotes for Hb Hasharon and β-thalassemia from 43 apparently unrelated families living in the province of Rovigo (northern Italy). Biosynthetic studies are also reported. The data strongly suggest the presence of an α-thalassemia-2 determinant closely linked to the αHasharon-chain locus. Selective advantage of heterozygotes carrying such a-haplotype would explain the relatively high frequency of Hb Hasharon (0.23%) in northeastern Italy, a past-endemic malaria region. The interaction between Hb Hasharon and β-thalassemia results in preferential decrease of the abnormal Hb level. © 1980 S. Karger AG, Basel.
Hemoglobin hasharon [α2 47 (CD5) Asp→His β2] linked to α-thalassemia in northern italian carriers: Hematological and biosynthetic studies / Mavilio, F.; Marinucci, M.; Massa, A.; Fontanarosa, P. P.; Tentori, L.; Cappellozza, G.. - In: ACTA HAEMATOLOGICA. - ISSN 0001-5792. - 63:6(1980), pp. 305-311. [10.1159/000207426]
Hemoglobin hasharon [α2 47 (CD5) Asp→His β2] linked to α-thalassemia in northern italian carriers: Hematological and biosynthetic studies
Mavilio F.;
1980
Abstract
This report is concerned with the evaluation of hematological parameters and of both relative (%) and absolute (mean pg/cell) quantities of the abnormal Hemoglobin (Hb) Hasharon in 53 heterozygous carriers and 7 double heterozygotes for Hb Hasharon and β-thalassemia from 43 apparently unrelated families living in the province of Rovigo (northern Italy). Biosynthetic studies are also reported. The data strongly suggest the presence of an α-thalassemia-2 determinant closely linked to the αHasharon-chain locus. Selective advantage of heterozygotes carrying such a-haplotype would explain the relatively high frequency of Hb Hasharon (0.23%) in northeastern Italy, a past-endemic malaria region. The interaction between Hb Hasharon and β-thalassemia results in preferential decrease of the abnormal Hb level. © 1980 S. Karger AG, Basel.
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