TY - JOUR
T1 - β-Thalassemia and Polycythemia vera
T2 - Targeting chronic stress erythropoiesis
AU - Crielaard, Bart J
AU - Rivella, Stefano
N1 - Copyright © 2014 Elsevier Ltd. All rights reserved.
PY - 2014/6
Y1 - 2014/6
N2 - 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.
AB - 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.
U2 - 10.1016/j.biocel.2014.03.029
DO - 10.1016/j.biocel.2014.03.029
M3 - Article
C2 - 24718374
SN - 1357-2725
VL - 51
SP - 89
EP - 92
JO - International journal of biochemistry & cell biology
JF - International journal of biochemistry & cell biology
ER -