African Journal of
Biotechnology

  • Abbreviation: Afr. J. Biotechnol.
  • Language: English
  • ISSN: 1684-5315
  • DOI: 10.5897/AJB
  • Start Year: 2002
  • Published Articles: 12501

Full Length Research Paper

Erythroblast cell expansion as a marker for disease severity in b0-thalassemia/Hb E disease

  Amporn Leecharoenkiat, Tirawat Wannatung and Duncan R. Smith*  
Molecular Pathology Laboratory Institute of Molecular Biosciences, Mahidol University, Thailand.
Email: [email protected]

  •  Accepted: 22 June 2011
  •  Published: 05 January 2012

Abstract

 

b0-Thalassemia/Hb E disease is extremely common in many parts of Southeast Asia.  In Thailand, there are believed to be approximately 100,000 people living with this disease which can present as a life threatening anemia syndrome dependent on regular blood transfusions. Determination of the state of presentation of the disease (mild, moderate or severe) is based upon a number of clinical and hematological parameters and currently, there is no accurate predictive methodology to gauge the final severity of the disease in a particular patient. While erythropoiesis, the process generating red blood cells, takes place in the bone marrow, sufficient circulating erythroid progenitor cells are found in the peripheral blood circulation to provide materials for analysis. In this study, the expansion of isolated erythroid progenitor cells was analyzed in culture and the level of expansion assessed in relation to disease severity. A clear statistically significant relationship between erythroid progenitor expansion and disease severity was observed, providing for the first time a potential predictive marker for disease severity for b0-thalassemia/Hb E disease.

 

Key words: β-Thalassemia, biomarker, erythroblasts, hemoglobin E, severity.