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β-Thalassemia and Polycythemia vera: targeting chronic stress erythropoiesis.

Authors :
Crielaard BJ
Rivella S
Source :
The international journal of biochemistry & cell biology [Int J Biochem Cell Biol] 2014 Jun; Vol. 51, pp. 89-92. Date of Electronic Publication: 2014 Apr 06.
Publication Year :
2014

Abstract

β-Thalassemia and Polycythemia vera are genetic disorders which affect the synthesis of red blood cells, also referred to as erythropoiesis. Although essentially different in clinical presentation - patients with β-thalassemia have an impairment in β-globin synthesis leading to defective erythrocytes and anemia, while patients with Polycythemia vera present with high hemoglobin levels because of excessive red blood cell synthesis - both pathologies may characterized by lasting high erythropoietic activity, i.e. chronic stress erythropoiesis. In both diseases, therapeutic strategies targeting chronic stress erythropoiesis may improve the address phenotype and prevent secondary pathology, such as iron overload. The current review will address the basic concepts of these strategies to reduce chronic stress erythropoiesis, which may have significant clinical implications in the near future.<br /> (Copyright © 2014 Elsevier Ltd. All rights reserved.)

Details

Language :
English
ISSN :
1878-5875
Volume :
51
Database :
MEDLINE
Journal :
The international journal of biochemistry & cell biology
Publication Type :
Academic Journal
Accession number :
24718374
Full Text :
https://doi.org/10.1016/j.biocel.2014.03.029