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Widely distribution of hematological parameters in thalassemia patients with similar α-globin genotype

Frontiers in Biology 2018年第6期13卷 469-474页

作者： Bijan Keikhaei Pejman Salehi-Fard Mostafa Paridar Mehraneh Karimzadeh Razie Dehghani Asma Zamiri Vahideh Takhviji Research Center for thalassemia and Hemoglobinopathy Health InstituteAhvaz Jundishapur University of Medical SciencesAhvazIran Deputy of Management and Resources Development Ministry of Health and Medical EducationTehranIran School of Medicine Shahid Beheshti University of Medical SciencesTehranIran Pediatric Department Ahvaz Jundishapur University of Medical SciencesAhvazIran School of Medicine Gorgan University of Medical SciencesGorganIran Laboratory Hematology and BloodBanla'ng School of Allied Medical ScienceShahid Beheshti University of Medical ScienceTehranIran

Background thalassemia is known as the commonest monogenic disorder with an imbalanced rate of globin chains production of adult hemoglobin. Despite the available information about the thalassemia etiology, its phenotype varies from each patient to another. This study aimed to evaluate the hematological parameters of patients with the same -α3.7 homozygote and heterozygote genotypes to amend screening *** In this observational study, we evaluated 1301 thalassemia suspected patients who referred to the thalassemia and Hemoglobinopathy Research Center of Ahvaz University of Medical Sciences, Khuzestan, Iran during 2014-2016. According to the genotyping studies, patients divided into 2 groups with -α3.7/αα (n = 646) and -α3.7/-α3.7 (n = 181) genotypes. Thereafter, distribution of hematological parameters evaluated in both *** The mean age in heterozygous and homozygous groups was 25.7±4.5 and 26±4.4 years old, respectively. The degree of anemia was considerably varied in patients with the same genotype. MCV, RBC and MCH showed a wide distribution in *** The findings presented here suggest that other molecular mechanisms along with α-globin gene mutations could be involved in determining the phenotypes of alpha thalassemia patients.

关键词： hematological parameters α-globin genotype alpha thalassemia

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