Publication: Accelerated telomere shortening in β-thalassemia/HbE patients
Issued Date
2015-08-01
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ISSN
10960961
10799796
10799796
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2-s2.0-84933557244
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Mahidol University
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SCOPUS
Bibliographic Citation
Blood Cells, Molecules, and Diseases. Vol.55, No.2 (2015), 173-179
Suggested Citation
Pornthip Chaichompoo, Kovit Pattanapanyasat, Pranee Winichagoon, Suthat Fucharoen, Saovaros Svasti Accelerated telomere shortening in β-thalassemia/HbE patients. Blood Cells, Molecules, and Diseases. Vol.55, No.2 (2015), 173-179. doi:10.1016/j.bcmd.2015.01.003 Retrieved from: https://repository.li.mahidol.ac.th/handle/20.500.14594/35410
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Title
Accelerated telomere shortening in β-thalassemia/HbE patients
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Abstract
© 2015 Elsevier Inc. β-Thalassemia/HbE disease is caused by a defective β-globin synthesis that leads to accumulation of excess unbound α-globins, and consequently oxidative stress, ineffective erythropoiesis and chronic anemia. Cell replication and oxidative stress are factors contributing to erosion of telomeres responsible for maintaining genomic stability and cell replication capability. In this study, the rate of telomere shortening in β-thalassemia/HbE patients was compared to the rate of telomere shortening in normal individuals. Telomere length was determined from peripheral blood mononuclear cells of 43 β-thalassemia/HbE patients and 22 normal controls using Flow-FISH analysis. The telomere length was shown to be age-dependent in normal group (rs=0.715, P=0.002), whereas severity-dependent telomere shortening was observed in the patients. The telomere length of patients who had severe clinical symptoms (10.07±2.15%) was shorter than that of patients who showed mild symptoms (15.59±2.27%), moderate symptoms (14.50±1.41%) and those in the normal group (14.75±3.11%, P<0.05). Additionally, reticulocyte count and oxidative stress were correlated with telomere length. This indicates that increased oxidative stress and markedly enhanced erythropoiesis in β-thalassemia/HbE patients leads to accelerated telomere erosion in clinically severe patients.