Publication: Hydroxyurea responses and fetal hemoglobin induction in β-thalassemia/HbE patients' peripheral blood erythroid cell culture
Issued Date
2006-03-01
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ISSN
09395555
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2-s2.0-31744445922
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Mahidol University
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SCOPUS
Bibliographic Citation
Annals of Hematology. Vol.85, No.3 (2006), 164-169
Suggested Citation
Ramida Watanapokasin, Duangmanee Sanmund, Pranee Winichagoon, Koichiro Muta, Suthat Fucharoen Hydroxyurea responses and fetal hemoglobin induction in β-thalassemia/HbE patients' peripheral blood erythroid cell culture. Annals of Hematology. Vol.85, No.3 (2006), 164-169. doi:10.1007/s00277-005-0049-1 Retrieved from: https://repository.li.mahidol.ac.th/handle/20.500.14594/23809
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Title
Hydroxyurea responses and fetal hemoglobin induction in β-thalassemia/HbE patients' peripheral blood erythroid cell culture
Abstract
Due to genetic heterogeneity of β-thalassemia (β-thal) patients, several efforts have been undertaken to determine the efficacy of hydroxyurea treatment. The aim of this work is to determine the responder and nonresponder for hydroxyurea treatment in β-thal intermedia based on γ-globin mRNA and fetal hemoglobin (HbF) induction in human erythroid progenitor cells purified from a patient's peripheral blood. Eighteen β-thal/hemoglobin E patients [13 βE/codon41/42(-TCTT), 4 βE/codon17, and 1 βE/IVS-654], requiring blood transfusion occasionally, with Hb levels of 5.20-8.50 g/dl were studied. The relative levels of γ-globin mRNA was measured by real-time reverse-transcription polymerase chain reaction and HbF by high-performance liquid chromatography. The results indicated that erythroid progenitor cells treated with 30 μmol/l hydroxyurea for 96 h preferentially enhancedGγ-andAγ-globin mRNA. The mean values ofGγ-globin mRNA fold induction were higher thanAγ-globin mRNA (12±4 vs 4±0.30), the Pearson's correlation ofGγ-andAγ- globin mRNA was r=0.80. Induction ofGγ/Aγ globin mRNA is up to ninefold. A 30% increase in the proportion of HbF out of the total Hb was found in cultures derived from four patients, 20-30% in cultures from nine patients, and less than 20% in cultures from five patients. In cultures from only two patients, increase in the proportion of HbF was less than 3%, andGγ/Aγ globin mRNA is less than 0.50. © Springer-Verlag 2006.