The Interplay between Drivers of Erythropoiesis and Iron Homeostasis in Rare Hereditary Anemias: Tipping the Balance
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 d...
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MDPI AG
2021-02-01
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| Series: | International Journal of Molecular Sciences |
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| Online Access: | https://www.mdpi.com/1422-0067/22/4/2204 |
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doaj-a668a767227c4e03a1f74ddefe4c6b0a2021-02-24T00:02:42ZengMDPI AGInternational Journal of Molecular Sciences1661-65961422-00672021-02-01222204220410.3390/ijms22042204The Interplay between Drivers of Erythropoiesis and Iron Homeostasis in Rare Hereditary Anemias: Tipping the BalanceSimon Grootendorst0Jonathan de Wilde1Birgit van Dooijeweert2Annelies van Vuren3Wouter van Solinge4Roger Schutgens5Richard van Wijk6Marije Bartels7Department of Clinical Chemistry and Haematology, University Medical Center Utrecht, 3584 CX Utrecht, The NetherlandsDepartment of Clinical Chemistry and Haematology, University Medical Center Utrecht, 3584 CX Utrecht, The NetherlandsDepartment of Clinical Chemistry and Haematology, University Medical Center Utrecht, 3584 CX Utrecht, The NetherlandsVan Creveldkliniek, University Medical Center Utrecht, 3584 CX Utrecht, The NetherlandsDepartment of Clinical Chemistry and Haematology, University Medical Center Utrecht, 3584 CX Utrecht, The NetherlandsVan Creveldkliniek, University Medical Center Utrecht, 3584 CX Utrecht, The NetherlandsDepartment of Clinical Chemistry and Haematology, University Medical Center Utrecht, 3584 CX Utrecht, The NetherlandsVan Creveldkliniek, University Medical Center Utrecht, 3584 CX Utrecht, The NetherlandsRare 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.https://www.mdpi.com/1422-0067/22/4/2204erythropoiesisiron metabolismiron overloadineffective erythropoiesis |
| collection |
DOAJ |
| language |
English |
| format |
Article |
| sources |
DOAJ |
| author |
Simon Grootendorst Jonathan de Wilde Birgit van Dooijeweert Annelies van Vuren Wouter van Solinge Roger Schutgens Richard van Wijk Marije Bartels |
| spellingShingle |
Simon Grootendorst Jonathan de Wilde Birgit van Dooijeweert Annelies van Vuren Wouter van Solinge Roger Schutgens Richard van Wijk Marije Bartels The Interplay between Drivers of Erythropoiesis and Iron Homeostasis in Rare Hereditary Anemias: Tipping the Balance International Journal of Molecular Sciences erythropoiesis iron metabolism iron overload ineffective erythropoiesis |
| author_facet |
Simon Grootendorst Jonathan de Wilde Birgit van Dooijeweert Annelies van Vuren Wouter van Solinge Roger Schutgens Richard van Wijk Marije Bartels |
| author_sort |
Simon Grootendorst |
| title |
The Interplay between Drivers of Erythropoiesis and Iron Homeostasis in Rare Hereditary Anemias: Tipping the Balance |
| title_short |
The Interplay between Drivers of Erythropoiesis and Iron Homeostasis in Rare Hereditary Anemias: Tipping the Balance |
| title_full |
The Interplay between Drivers of Erythropoiesis and Iron Homeostasis in Rare Hereditary Anemias: Tipping the Balance |
| title_fullStr |
The Interplay between Drivers of Erythropoiesis and Iron Homeostasis in Rare Hereditary Anemias: Tipping the Balance |
| title_full_unstemmed |
The Interplay between Drivers of Erythropoiesis and Iron Homeostasis in Rare Hereditary Anemias: Tipping the Balance |
| title_sort |
interplay between drivers of erythropoiesis and iron homeostasis in rare hereditary anemias: tipping the balance |
| publisher |
MDPI AG |
| series |
International Journal of Molecular Sciences |
| issn |
1661-6596 1422-0067 |
| publishDate |
2021-02-01 |
| description |
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. |
| topic |
erythropoiesis iron metabolism iron overload ineffective erythropoiesis |
| url |
https://www.mdpi.com/1422-0067/22/4/2204 |
| work_keys_str_mv |
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