Ineffective Erythropoiesis: Associated Factors and Their Potential as Therapeutic Targets in  Beta-Thalassaemia Major

Heba Alsaleh; Sarina Sulong; Bin Alwi Zilfalil; Rosline Hassan

doi:10.9734/BJMMR/2017/31489

Research Article Open access CC BY 4.0

Ineffective Erythropoiesis: Associated Factors and Their Potential as Therapeutic Targets in Beta-Thalassaemia Major

Heba Alsaleh, Sarina Sulong, Bin Alwi Zilfalil, Rosline Hassan

Journal of Advances in Medicine and Medical Research · pp. 1–9 · Published 28 Apr 2017

10.9734/BJMMR/2017/31489

Abstract

Beta-thalassaemia (β-thal.) is single-gene disorder that exhibits much clinical variability. β-thal. major is a major health problem, and the only method of curing is allogenic bone-marrow transplantation, which is not available to everyone and not without risk. The underlying pathogenesis of β-thal. major is due to ineffective erythropoiesis (IE), which is characterized by increased proliferative activity that fails to produce sufficient functional red blood cells. In β-thal. patients, the severity of the IE is mainly responsible for the hallmarks of the disease’s presentation, sequalae and complications. This review discusses the mechanisms of IE, the factors that contribute to it and the potential therapies for targeting these factors to improve patients’ clinical phenotypes.

Thalassaemia ineffective erythropoiesis gene therapy apoptosis

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Heba Alsaleh, Sarina Sulong, Bin Alwi Zilfalil, Rosline Hassan (2017). Ineffective Erythropoiesis: Associated Factors and Their Potential as Therapeutic Targets in Beta-Thalassaemia Major. Journal of Advances in Medicine and Medical Research , 1–9. https://doi.org/10.9734/BJMMR/2017/31489

Affiliations

Heba Alsaleh — Department of Haematology, School of Medical Sciences, Universiti Sains Malaysia, Health Campus, 16150, Kubang Kerian, Kelantan, Malaysia.
Sarina Sulong — Human Genome Centre, School of Medical Sciences, Universiti Sains Malaysia, Health Campus, 16150, Kubang Kerian, Kelantan, Malaysia.
Bin Alwi Zilfalil — Department of Paediatrics, School of Medical Sciences, Universiti Sains Malaysia, 16150, Kubang Kerian, Kelantan, Malaysia.
Rosline Hassan — Department of Haematology, School of Medical Sciences, Universiti Sains Malaysia, Health Campus, 16150, Kubang Kerian, Kelantan, Malaysia.

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