Abstract
Usually sickle cell traits are asymptomatic but co-existence of various factrors may alter the clinical as well as biochemical levels. In India sickle cell traits are neglected condition. Here we are presenting the alpha deletion in association with low serum iron and increased HbF level with Xmn-1 carriers in sickle cell traits. Sickle traits with alpha deletions had significantly low level of serum iron (P-value <0.05) with low level of reticulocytes and red cell indices while Xmn-1 polymorphism associated with increased HbF level. Study concludes low serum iron associated with alpha deletions and high level of HbF associated with Xmn-1 polymorphism in sickle cell traits.
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Eichner ER. Sickle cell trait. J Sport Rehabil. 2007;16(3):197–203.
Tsaras G, Owusu-Ansah A, Boateng FO, Amoateng-Adjepong Y. Complications associated with sickle cell trait: a brief narrative review. Am J Med. 2009;122(6):507–12.
Kanavakis E, Wainscoat JS, Wood WG, Weatherall DJ, Cao A, Furbetta M, et al. The intraction of alpha thalassemia with hetrozygus beta thalassemia. Br J Haematol. 1982;52(3):465–73.
Wainscoat JS, Kanavakis E, Wood WG, Letsky EA, Huehns ER, Marsh GW, Higgs DR, et al. Thalassemia intermedia in Cyprus: the interaction of alpha and beta thalassemia. Br J Haematol. 1983;53(3):411–6.
Gilman JG, Huisman THJ. DNA sequence variation associated with elevated fetal Gg globin production. Blood. 1985;66:783–7.
Dedoussis GV, Mandilara GD, Boussiv M, Loutradis A. HbF production in b-thalassaemia heterozygotes for the IVSII-1 G-A β0-globin mutation. Implication of the haplotype and the Gg-158 C-T mutation on the HbF level. Am J Hematol. 2000;64(3):151–5.
Waterfall CM, Benjamin DC. Single tube genotyping of sickle cell anaemia using PCR-based SNP analysis. Nucleic Acids Res. 2001;29(23):e119.
Baysal E, Huisman THJ. Detection of common deletional alpha thalassemia-2 determinants by GAP-PCR. Am J Hematol. 1994;46:208–13.
Smetanina NS, Huisman TH. Detection of alpha-thalassemia-2 (−3.7 kb) and its corresponding triplication ααα (anti-3.7 kb) by PCR: an improved technical change. Am J Hematol. 1996;53(3):202–3.
Shaji RV, Eunice SE, Baidya S, Srivastava A, Chandy M. Determination of the breakpoint and molecular diagnosis of a common alpha-thalassaemia-1 deletion in the Indian population. Br J Haematol. 2003;123(5):942–7.
Chang JG, Lee LS, Lin CP, Chen PH, Chen CP. Rapid diagnosis of alpha–thalassemia-1 of Southeast Asia type and hydrops fetailis by polymerase chain reaction. Blood. 1991;78:853–4.
Sutton M, Bouhassira EE, Nagel RL. Polymerase chain reaction amplification applied to the determination of beta like globin gene cluster haplotypes. Am J Hematol. 1989;32:66–9.
Morris CR. Mechanisms of vasculopathy in sickle cell disease and thalassemia. Hematol Am Soc Hematol Educ Progr. 2008;177–185.
Stuart MJ, Nagel RL. Sickle-cell disease. Lancet. 2004;364:1343–60.
Koduri PR. Iron in sickle cell disease: a review why less is better. Am J Hematol. 2003;73:59–63.
Raffaella M, Paola T, Matteo P, Alberto P. Iron metabolism in thalassemia and sickle cell disease. Medit J Hemat Infect Dis. 2009;1(1): Open Journal System.
Julius B, Louis JF. Sickle cell disease: with special regard to its non anemic variety. Arch Surg. 1943;47(6):553–63.
Nava MP, Ibarra B, Magaña MT, de la Luz Chávez M, Perea FJ. Prevalence of −α3.7 and αααanti3.7 alleles in sickle cell trait and β-thalassemia patients in Mexico. Blood Cells Mol Dis. 2006;36(2):2255–8.
Higgs DR, Aldridge BE, Lamb J, Clegg JB, Weatherall DJ, Hayes RJ, Grandison Y, et al. The interaction of alpha thalassemia and homozygous sickle cell disease. N Eng J Med N Engl J Med. 1982;306(24):1441–6.
Embury SH, Dozy AM, Miller J, Davis JR, Kleman KM, Preisler H, et al. Concurrent sickle cell anemia and alpha thalassemia. Effect on severity. N Engl J Med. 1982;306(5):270–4.
de Ceulaer K, Higgs DR, Weatherall DJ, Hayes RJ, Serjeant BE, Serjeant GR. Alpha thalassemia reduces the hemolytic rate in homozygous sickle cell disease. N Engl J Med. 1983;309(3):189–90.
Garner C, Tatu T, Reittie JE, Littlewood T, Darley J, Cervino S, et al. Genetic influences on F cells and other hematologic variables: a twin heritability study. Blood. 2000;95:342–6.
Serjeant GR. Sickle cell- β thalassemia. In: Serjeant GR, editor. Sickle Cell Disease. 3rd ed. Oxford: Oxford University Press; 2001.
Panigrahi I, Agarwal S, Gupta T, Singhal P, Pradhan M. Hemoglobin E-beta thalassemia: factors affecting phenotype. Indian Pediatr. 2005;42:357–62.
Shimmoto MM, Vicari P, Fernandes AC, Guimaraes GS, Figueiredo MS. XmnI polymorphism is associated with fetal hemoglobin levels in hypoplastic syndromes. Sao Paulo Med J. 2006;124(2):110–1.
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Pandey, S., Mishra, R.M., Suhail, A. et al. Association of Low Serum Iron with Alpha Globin Gene Deletions and High Level of HbF with Xmn-1 Polymorphism in Sickle Cell Traits. Ind J Clin Biochem 27, 270–273 (2012). https://doi.org/10.1007/s12291-011-0170-y
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DOI: https://doi.org/10.1007/s12291-011-0170-y