Ineffective Erythropoiesis: Associated Factors and Their Potential as Therapeutic Targets in Beta-Thalassaemia Major
Journal Title: Journal of Advances in Medicine and Medical Research - Year 2017, Vol 21, Issue 1
Abstract
Beta-thalassaemia (-thal.) is single-gene disorder that exhibits much clinical variability. -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 -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 -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.
Authors and Affiliations
Heba Alsaleh, Sarina Sulong, Bin Alwi Zilfalil, Rosline Hassan
Keywords
Related Articles
- EP ID EP311755
- DOI 10.9734/BJMMR/2017/31489
- Views 103
- Downloads 0
Correction of Skeletal Openbite Using Zygomatic Miniplates
Aims: Anterior open bite is often caused by excessive vertical development of the posterior maxilla. In such cases, it is hardly possible to establish absolute anchorage for molar intrusion by traditional orthodontic mec...
The Inductive Effect of Purified Streptococcal M-protein on Immunity against Streptococcus pyogenes in Rabbit Model
Aims: The study aimed to know the effects of the purified M-protein on immune system to produce protection against Streptococcus pyogenes in rabbits. Study Design: Case-control study. Place and Duration of Study: In this...
Isoflurane and Ketamine/Xylazine Anesthesia do not Influence the Neuroprotective Effects of Simvastatin in a Model of Permanent Cerebral Ischemic Injury in C57BL/6J Mice
Laboratory mice with Middle Cerebral Artery Occlusion (MCAO) represent the main animal models for ischemic stroke research. Appropriate anesthetic protocols are essential, as anesthetic agents might affect the central ne...
Short Term Effects of Islamic Zikr on Anxiety, Stress, and Depression in Mothers of Children with Congenital Heart Disease
Aim: We investigated immediate short- term effect of Islamic Allah’s remembrance on depression, stress and anxiety of mothers of the CHD patients. Study Design and Methodology: The Depression, Anxiety, and Stress Scales...
s-Ethyl Cysteine and s-Methyl Cysteine Post-intake Attenuated LPS-induced Lung Injury in Mice
Objective: Effects of s-ethyl cysteine (SEC) and s-methyl cysteine (SMC) upon lipopolysaccharide (LPS)-induced acute lung injury in mice were examined. Materials and Methods: The acute lung injury was induced by intrana...