Hemoglobinopathies in Iran: An Updated Review
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Abstract
Hemoglobinopathies are the most common single gene disorders (monogenic disorders) in the world population. Due to specific position of Iran and the presence of multi-ethnic groups in the country, there are many varieties in the molecular genetics and clinical features of hemoglobinopathies in Iran. Hemoglobinopathies include structural variants, thalassemias, and hereditary persistence of fetal hemoglobin. In this review, we look at the common structural variants in various parts of the country along with their hematological and clinical characteristics. Also, we discuss about the burden of the thalassemias in the country, different types, complications, molecular defects and therapy.
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2. Weatherall D. Current trends in the diagnosis and management of haemoglobinopathies. Scand J Clin Lab Invest. 2007; 67(1):1-2.
3. Rahimi Z. Genetic epidemiology, hematological and clinical features of hemoglobinopathies in Iran. BioMed Res Int. 2013; 2013:1-10.
4. Acquaye JK, Omer A, Ganeshaguru K, et al. Non‐benign sickle cell anaemia in western Saudi Arabia. Br J Haematol. 1985; 60(1):99-108.
5. Adekile A, Haider M. Morbidity, βS haplotype and α-globin gene patterns among sickle cell anemia patients in Kuwait. Acta Haematol. 1996; 96(3):150-4.
6. Adekile A. Historical and anthropological correlates of βS haplotypes and α-and β-thalassemia alleles in the Arabian Peninsula. Hemoglobin. 1997; 21(3):281-96.
7. Al Arrayed SS, Haites N. Features of sickle-cell disease in Bahrain. 1995; 12(4):41-48.
8. Zamani S, Borhan Haghighi A, Haghpanah S, et al. Transcranial Doppler Screening in 50 Patients With Sickle Cell Hemoglobinopathies in Iran. J Pediatr Hematol Oncol. 2017; 39(7):506-512.
9. Alsultan A, Aleem A, Ghabbour H, et al. Sickle cell disease subphenotypes in patients from Southwestern Province of Saudi Arabia. J Pediatr Hematol Oncol. 2012; 34(2):79-84.
10. Haghshenass M, Ismail-Beigi F, Clegg J, et al. Mild sickle-cell anaemia in Iran associated with high levels of fetal haemoglobin. J Med Genet. 1977; 14(3):168-171.
11. Habibzadeh F, Yadollahie M, Ayatollahie M, et al. The prevalence of sickle cell syndrome in south of Iran. Iran J Med Sci. 1999;24:32-4.
12. Rahgozar S, Poorfathollah AA, Moafi AR, et al. βS gene in Central Iran is in linkage disequilibrium with the Indian–Arab haplotype. Am J Hematol. 2000; 65(3):192-195.
13. Booth C, Inusa B, Obaro SK. Infection in sickle cell disease: a review. Int J Infect Dis. 2010; 14(1):e2-e12.
14. Keikhaei B, Mohseni AR, Norouzirad R, A, et al. Altered levels of pro-inflammatory cytokines in sickle cell disease patients during vaso-occlusive crises and the steady state condition. Eur Cytokine Netw. 2013; 24(1):45-52.
15. Shahripour RB, Mortazavi MM, Barlinn K, et al. Can stop trial velocity criteria be applied to Iranian children with sickle cell disease? J stroke. 2014; 16(2):97-101.
16. Green NS, Barral S. Emerging science of hydroxyurea therapy for pediatric sickle cell disease. Pediatr Res. 2014; 75(0):196-204.
17. Keikhaei B, Yousefi H, Bahadoram M. Hydroxyurea: Clinical and hematological effects in patients with sickle cell anemia. Glob J Health Sci. 2016; 8(3):252-256.
18. Rahimi Z, Merat A, Gerard N, et al. Implications of the genetic epidemiology of globin haplotypes linked to the sickle cell gene in southern Iran. Hum Biol. 2006:78 (6):719-31.
19. Rusanova I, Cossio G, Moreno B, et al. β‐globin gene cluster haplotypes in sickle cell patients from Panamá. Am J Hum Biol. 2011; 23(3):377-80.
20. Rahimi Z, Karimi M, Haghshenass M, et al. β‐Globin gene cluster haplotypes in sickle cell patients from southwest Iran. Am J Hematol. 2003;74(3):156-60.
21. Rahimi Z, Vaisi-Raygani A, Merat A, et al. Level of hemoglobin F and Gg gene expression in sickle cell disease and their association with haplotype and XmnI polymorphic site in South of Iran. Iran J Med Sci. 2007; 32(4):234-239.
22. Rahimi Z, Muniz A, Mozafari H. Abnormal hemoglobins among Kurdish population of Western Iran: hematological and molecular features. Mol Biol Rep. 2010; 37(1):51-7.
23. Rahimi Z, Akramipour R, Nagel RL, et al. The β-globin gene haplotypes associated with Hb D-Los Angeles [β121 (GH4) Glu→ Gln] in western Iran. Hemoglobin. 2006;30(1):39-44.
24. Galehdari H, Salehi B, Azmoun S, et al. Comprehensive spectrum of the β-thalassemia mutations in Khuzestan, Southwest Iran. Hemoglobin. 2010;34(5):461-68.
25. Bunn HF, Forget BG. Hemoglobin: molecular, genetic, and clinical aspects. WB Saunders Co.; 1986; 35(1):26-54.
26. Aghdashloo BE, Khmenini O, Shohreh P. Co-legacy against 3.7 triplication with hemoglobin D/0 thalassemia: A case report from South west of Iran. Int J Genet. Genomics. 2015;2 (3):080-084
27. Yavarian M, Karimi M, Paran F, et al. Multi Centric Origin of Hb D-Punjab [β121 (GH4) Glu→ Gln, G AA> C AA]. Hemoglobin. 2009;33(6):399-405.
28. Mahdavi MR, Jalali H, Kosaryan M, et al. β-Globin gene cluster haplotypes of Hb D-Los Angeles in Mazandaran province, Iran. Genes Genet Syst. 2015; 90(1):55-57.
29. Lorkin P, Charlesworth D, Lehmann H, et al. Two haemoglobins Q, α74 (EF3) and α75 (EF4) Aspartic acid→ Histidine. Br J Haematol. 1970; 19(1):117-125.
30. Aksoy M, Gurgey A, Altay C, et al. Some notes about Hb Q-India and Hb Q-Iran. Hemoglobin. 1986; 10(2):215-9.
31. Khorshidi M, Roshan P, Bayat N, et al. Hemoglobin Q-Iran detected in family members from Northern Iran: a case report. J Med Case Reports. 2012; 6: 47.
32. Rahimi Z, Akramipour R, Vaisi-Raygani A, et al. An Iranian Child With HbQ-Iran [α75 (EF4) Asp→ His]/− α3. 7 kb/IVSII. 1 G→ A: First Report. J Pediatr Hematol Oncol. 2007; 29(9):649-651.
33. Rahimi Z, Rezaei M, Nagel RL, Muniz A. Molecular and hematologic analysis of hemoglobin Q-Iran and hemoglobin Setif in Iranian families. Arch Iran Med. 2008;11(4):382-6.
34. Nozari G, Ralthar S, Darbre P, et al. Hemoglobin Setif (α94 (G1) ASP→ TYR) in Iran a report of 9 Cases. Hemoglobin. 1977; 1(3):289-292.
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| Files | ||
| Issue | Vol 14, No 2 (2020) | |
| Section | Review Article(s) | |
| DOI | https://doi.org/10.18502/ijhoscr.v14i2.2679 | |
| Keywords | ||
| Hemoglobinopathies; Thalassemia, HbS, HbD, Mutation | ||
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This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License. |
How to Cite
1.
Nasiri A, Rahimi Z, Vaisi-Raygani A. Hemoglobinopathies in Iran: An Updated Review. Int J Hematol Oncol Stem Cell Res. 2020;14(2):140-150.
