1. The Interplay between Drivers of Erythropoiesis and Iron Homeostasis in Rare Hereditary Anemias: Tipping the Balance
- Author
-
Birgit van Dooijeweert, Simon T. Grootendorst, Jonathan de Wilde, Richard van Wijk, Roger E. G. Schutgens, Wouter W. van Solinge, Marije Bartels, and Annelies J. Van Vuren
- Subjects
Ineffective erythropoiesis ,medicine.medical_specialty ,Iron ,Review ,medicine.disease_cause ,Catalysis ,lcsh:Chemistry ,Inorganic Chemistry ,03 medical and health sciences ,0302 clinical medicine ,Iron homeostasis ,Internal medicine ,medicine ,Homeostasis ,Humans ,iron metabolism ,In patient ,iron overload ,Physical and Theoretical Chemistry ,lcsh:QH301-705.5 ,Molecular Biology ,Spectroscopy ,ineffective erythropoiesis ,business.industry ,Organic Chemistry ,Anemia ,General Medicine ,medicine.disease ,Hemolysis ,Computer Science Applications ,Endocrinology ,lcsh:Biology (General) ,lcsh:QD1-999 ,030220 oncology & carcinogenesis ,Transfusion dependence ,Erythropoiesis ,business ,Chronic anemia ,erythropoiesis ,030215 immunology - Abstract
Rare hereditary anemias (RHA) represent a group of disorders characterized by either impaired production of erythrocytes or decreased survival (i.e., hemolysis). In RHA, the regulation of iron metabolism and erythropoiesis is often disturbed, leading to iron overload or worsening of chronic anemia due to unavailability of iron for erythropoiesis. Whereas iron overload generally is a well-recognized complication in patients requiring regular blood transfusions, it is also a significant problem in a large proportion of patients with RHA that are not transfusion dependent. This indicates that RHA share disease-specific defects in erythroid development that are linked to intrinsic defects in iron metabolism. In this review, we discuss the key regulators involved in the interplay between iron and erythropoiesis and their importance in the spectrum of RHA.
- Published
- 2021
- Full Text
- View/download PDF