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Hematol Oncol Clin North Am. 2018 Apr;32(2):213-221. doi: 10.1016/j.hoc.2017.11.009. Epub 2017 Dec 29.

Ineffective Erythropoiesis: Anemia and Iron Overload.

Author information

1
Department of Pediatrics, Division of Hematology, Children's Hospital of Philadelphia (CHOP), 3401 Civic Center Boulevard, Philadelphia, PA 19104, USA.
2
International Network of Hematology, London WC1V 6AX, UK.
3
Department of Internal Medicine, American University of Beirut Medical Center, PO Box: 11-0236, Cairo Street, Hamra, Raid E Solh, Beirut 1107 2020, Lebanon.
4
Department of Pediatrics, Division of Hematology, Children's Hospital of Philadelphia (CHOP), 3401 Civic Center Boulevard, Philadelphia, PA 19104, USA; Children's Hospital of Philadelphia (CHOP), Cell and Molecular Biology Graduate Group (CAMB), University of Pennsylvania, Abramson Research Center, 3615 Civic Center Boulevard, Room 316B, Philadelphia, PA 19104, USA. Electronic address: rivellas@email.chop.edu.

Abstract

Stress erythropoiesis (SE) is characterized by an imbalance in erythroid proliferation and differentiation under increased demands of erythrocyte generation and tissue oxygenation. β-thalassemia represents a chronic state of SE, called ineffective erythropoiesis (IE), exhibiting an expansion of erythroid-progenitor pool and deposition of alpha chains on erythrocyte membranes, causing cell death and anemia. Concurrently, there is a decrease in hepcidin expression and a subsequent state of iron overload. There are substantial investigative efforts to target increased iron absorption under IE. There are also avenues for targeting cell contact and signaling within erythroblastic islands under SE, for therapeutic benefits.

KEYWORDS:

Erythroblastic island; Hepcidin and iron homeostasis; Iron overload; Stress and ineffective erythropoiesis

PMID:
29458727
PMCID:
PMC5824437
[Available on 2019-04-01]
DOI:
10.1016/j.hoc.2017.11.009
[Indexed for MEDLINE]

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