β-Thalassemia and Polycythemia vera: targeting chronic stress erythropoiesis.
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.
Weill Cornell Medical College, Department of Pediatrics Hematology-Oncology, 515 E 71st Street, New York, 10021 NY, USA.
Bart J
BJ
Crielaard
Weill Cornell Medical College, Department of Pediatrics Hematology-Oncology, 515 E 71st Street, New York, 10021 NY, USA. Electronic address: str2010@med.cornell.edu.
Stefano
S
Rivella
D000818:Animals; D004920:Erythropoiesis / Q000502:physiology*; D006801:Humans; D011087:Polycythemia Vera / Q000097:blood*; D017086:beta-Thalassemia / Q000097:blood*
Chronic stress erythropoiesis; Iron metabolism; Macrophages; Polycythemia vera; β-Thalassemia