Publication:
Hydroxyurea responses and fetal hemoglobin induction in β-thalassemia/HbE patients' peripheral blood erythroid cell culture

0
0

Issued Date

2006

Resource Type

Article

File Type

application/pdf

ISSN

9395555

DOI

10.1007/s00277-005-0049-1

Other identifier(s)

2-s2.0-31744445922

Rights Holder(s)

Scopus

Bibliographic Citation

Annals of Hematology. Vol 85, No.3 (2006), p.164-169

Suggested Citation

Watanapokasin R., Sanmund D., Winichagoon P., Muta K., Fucharoen S. Hydroxyurea responses and fetal hemoglobin induction in β-thalassemia/HbE patients' peripheral blood erythroid cell culture. Annals of Hematology. Vol 85, No.3 (2006), p.164-169. doi:10.1007/s00277-005-0049-1 Retrieved from: https://hdl.handle.net/20.500.14740/5899

Title

Hydroxyurea responses and fetal hemoglobin induction in β-thalassemia/HbE patients' peripheral blood erythroid cell culture

Author(s)

Watanapokasin R.
 Sanmund D.
 Winichagoon P.
 Muta K.
 Fucharoen S.

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.

Subject(s)

Globin messenger RNA
 Hemoglobin E
 Hemoglobin F
 Hydroxyurea
 Article
 Beta thalassemia
 Blood transfusion
 Cell culture
 Clinical article
 Codon
 Controlled study
 Correlation analysis
 Drug mechanism
 Drug response
 Erythroid precursor cell
 Hemoglobin blood level
 High performance liquid chromatography
 Human
 Human cell
 Priority journal
 Real time polymerase chain reaction
 Reverse transcription polymerase chain reaction
 Beta-Thalassemia
 Blood Transfusion
 Cells, Cultured
 Chromatography, High Pressure Liquid
 Codon
 Dose-Response Relationship, Drug
 Enzyme Inhibitors
 Erythroid Progenitor Cells
 Erythropoiesis
 Female
 Fetal Hemoglobin
 Humans
 Hydroxyurea
 Male
 Reverse Transcriptase Polymerase Chain Reaction
 RNA, Messenger
 Up-Regulation

View online Resources

Research Projects

Organizational Units

Journal Issue

URI

https://hdl.handle.net/20.500.14740/5899

Collections

Scopus: Year 1983-2021

Endorsement

Review

Supplemented By

Referenced By