The interplay between drivers of erythropoiesis and iron homeostasis in rare hereditary anemias: Tipping the balance
Grootendorst, Simon; de Wilde, Jonathan; van Dooijeweert, Birgit; van Vuren, Annelies; van Solinge, Wouter; Schutgens, Roger; van Wijk, Richard; Bartels, Marije
(2021) International journal of molecular sciences, volume 22, issue 4
(Article)
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
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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.
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Keywords: Erythropoiesis, Ineffective erythropoiesis, Iron metabolism, Iron overload, Erythropoiesis/physiology, Homeostasis/physiology, Humans, Anemia/blood, Iron/metabolism, Iron Overload/diagnosis, iron overload, ineffective erythropoiesis, iron metabolism, erythropoiesis, Molecular Biology, Spectroscopy, Catalysis, Inorganic Chemistry, Computer Science Applications, Physical and Theoretical Chemistry, Organic Chemistry, Review, Journal Article
ISSN: 1661-6596
Publisher: Multidisciplinary Digital Publishing Institute (MDPI)
Note: Publisher Copyright: © 2021 by the authors. Licensee MDPI, Basel, Switzerland. Copyright: Copyright 2021 Elsevier B.V., All rights reserved.
(Peer reviewed)