, Volume 37, Issue 1, pp 51-57
Date: 31 Mar 2009

Abnormal hemoglobins among Kurdish population of Western Iran: hematological and molecular features

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Abstract

The type and frequency of structural hemoglobin variants and their hematological and molecular characteristics were identified using PCR-RFLP and sequencing techniques in 66 individuals from 33 unrelated families who referred to the two clinics of Kermanshah University of Medical Sciences from 2005 to 2006. We detected 28 subjects carrier for Hb D-Punjab (42.4%), 21 individuals carrier of Hb Q-Iran (31.8%), 12 subjects heterozygous for Hb Setif (18.2%), four cases with sickle cell disease (6.1%), and one case with Hb C (1.5%). All βS genes (4 genes) were linked to the Benin haplotype with negative Taq I site 5′ to γA gene. All βD-Punjab genes (29 genes) were in linkage disequilibrium with haplotype I. The only βC chromosome was linked to haplotype II. Both β0-thalassemia chromosomes with CD15 (G → A) mutation had haplotype background I. Three β+-thalassemia chromosomes with IVSI.110 (G → A) mutation were associated with haplotype I [+ − − − − + +]. In turn, the three β-thalassemia chromosomes with IVS II.1 G → A mutation were associated with atypical haplotype [− + + + + + −]. Hematological indices of carriers of Hb D-Punjab, Hb Q-Iran and Hb Setif were lower than those reported for normal individuals. For the first time, we have reported the haplotype background of βS gene among Kurdish population of Iran. Our results revealed that Hb D-Punjab is the most prevalent β-globin chain structural variant in this area and that is followed in frequency by an α-chain variant, Hb Q-Iran. The result of present study is useful for clinical management and the establishment of screening programmes in Western Iran.