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Frontiers in Biology

Front. Biol.    2018, Vol. 13 Issue (6) : 469-474     https://doi.org/10.1007/s11515-018-1522-2
RESEARCH ARTICLE
Widely distribution of hematological parameters in thalassemia patients with similar α-globin genotype
Bijan Keikhaei1, Pejman Salehi-Fard1, Mostafa Paridar2, Mehraneh Karimzadeh3, Razie Dehghani4, Asma Zamiri5, Vahideh Takhviji6()
1. Research Center for Thalassemia and Hemoglobinopathy, Health Institute, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran
2. Deputy of Management and Resources Development, Ministry of Health and Medical Education, Tehran, Iran
3. School of Medicine, Shahid Beheshti University of Medical Sciences, Tehran, Iran
4. Pediatric Department, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran
5. School of Medicine, Gorgan University of Medical Sciences, Gorgan, Iran
6. Laboratory Hematology and Blood Banking, School of Allied Medical Science, Shahid Beheshti University of Medical Science, Tehran, Iran
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Abstract

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 programs.

METHODS: 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/aa (n = 646) and -α3.7/-α3.7 (n = 181) genotypes. Thereafter, distribution of hematological parameters evaluated in both groups.

RESULTS: 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 patients.

CONCLUSION: 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.

Keywords hematological parameters      α-globin genotype      alpha thalassemia     
Corresponding Author(s): Vahideh Takhviji   
Online First Date: 23 October 2018    Issue Date: 30 November 2018
 Cite this article:   
Bijan Keikhaei,Pejman Salehi-Fard,Mostafa Paridar, et al. Widely distribution of hematological parameters in thalassemia patients with similar α-globin genotype[J]. Front. Biol., 2018, 13(6): 469-474.
 URL:  
http://journal.hep.com.cn/fib/EN/10.1007/s11515-018-1522-2
http://journal.hep.com.cn/fib/EN/Y2018/V13/I6/469
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Bijan Keikhaei
Pejman Salehi-Fard
Mostafa Paridar
Mehraneh Karimzadeh
Razie Dehghani
Asma Zamiri
Vahideh Takhviji
Alpha Mutation Age Hemoglobin MCV MCH RBC HbA2 HbF
-a3.7 Heterozygote
N = 646
25.73±4.58 13.16±1.49
9.8-18.5
77.68±4.45
59-88.5
25.01±1.72
16.2-30
5.29±0.58
3.95-7.15
2.44±0.37
1.4-3.3
0.56±0.41
0.0-2
-a3.7 Homozygote
N = 181
26.9±4.44 12.31±1.51
8-16.4
71.68±3.82
61.7-82.8
22.22±1.43
16.5-26.8
5.54±0.64
3.68-7.76
2.33±0.38
0.6-3.2
0.51±0.35
0.0-2.9
Tab.1  Hematological parameters
Fig.1  Hematological parameters distribution in patients harboring-a3.7/aa genotype.
Fig.2  Hematological parameters distribution in patients harboring -a3.7/-a3.7 genotype.
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