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Title: Hydroxyurea responses and fetal hemoglobin induction in β-thalassemia/HbE patients' peripheral blood erythroid cell culture
Authors: Ramida Watanapokasin
Duangmanee Sanmund
Pranee Winichagoon
Koichiro Muta
Suthat Fucharoen
Srinakharinwirot University
The Institute of Science and Technology for Research and Development, Mahidol University
Kyushu University, Faculty of Medical Sciences
Keywords: Medicine
Issue Date: 1-Mar-2006
Citation: Annals of Hematology. Vol.85, No.3 (2006), 164-169
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.
ISSN: 09395555
Appears in Collections:Scopus 2006-2010

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