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Title: Hydroxyurea responses and fetal hemoglobin induction in β-thalassemia/HbE patients' peripheral blood erythroid cell culture
Authors: Watanapokasin R.
Sanmund D.
Winichagoon P.
Muta K.
Fucharoen S.
Keywords: globin messenger RNA
hemoglobin E
hemoglobin F
beta thalassemia
blood transfusion
cell culture
clinical article
controlled study
correlation analysis
drug mechanism
drug response
erythroid precursor cell
hemoglobin blood level
high performance liquid chromatography
human cell
priority journal
real time polymerase chain reaction
reverse transcription polymerase chain reaction
Blood Transfusion
Cells, Cultured
Chromatography, High Pressure Liquid
Dose-Response Relationship, Drug
Enzyme Inhibitors
Erythroid Progenitor Cells
Fetal Hemoglobin
Reverse Transcriptase Polymerase Chain Reaction
RNA, Messenger
Issue Date: 2006
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 enhanced Gγ-and Aγ-globin mRNA. The mean values of Gγ-globin mRNA fold induction were higher than Aγ-globin mRNA (12±4 vs 4±0.30), the Pearson's correlation of Gγ-and Aγ- globin mRNA was r=0.80. Induction of Gγ/ 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%, and Gγ/Aγ globin mRNA is less than 0.50. © Springer-Verlag 2006.
ISSN: 9395555
Appears in Collections:Scopus 1983-2021

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