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Eur J Haematol. 2015 Jun;94(6):511-8. doi: 10.1111/ejh.12464. Epub 2014 Nov 11.

Altered erythropoiesis and iron metabolism in carriers of thalassemia.

Author information

1
Laboratório de Hematologia Clínica, Faculdade de Ciências Farmacêuticas de Ribeirão Preto, Universidade de São Paulo, Ribeirão Preto, Brazil.
2
The Strauss Thalassemia Laboratory, Pediatric Hematology-Oncology, Weill Medical College of Cornell University, New York, NY, USA.
3
Centro Regional de Hemoterapia de Ribeirão Preto, Departamento de Medicina Interna, Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo, Ribeirão Preto, Brazil.
4
Intrinsic LifeSciences, LLC, La Jolla, CA, USA.
5
New York Blood Center, New York, NY, USA.

Abstract

The thalassemia syndromes (α- and β-thalassemia) are the most common and frequent disorders associated with ineffective erythropoiesis. Imbalance of α- or β-globin chain production results in impaired red blood cell synthesis, anemia, and more erythroid progenitors in the blood stream. While patients affected by these disorders show definitive altered parameters related to erythropoiesis, the relationship between the degree of anemia, altered erythropoiesis, and dysfunctional iron metabolism has not been investigated in both α-thalassemia carriers (ATC) and β-thalassemia carriers (BTC). Here, we demonstrate that ATC have a significantly reduced hepcidin and increased soluble transferrin receptor levels but relatively normal hematological findings. In contrast, BTC have several hematological parameters significantly different from controls, including increased soluble transferrin receptor and erythropoietin levels. These changes in both groups suggest an altered balance between erythropoiesis and iron metabolism. The index sTfR/log ferritin and (hepcidin/ferritin)/sTfR are, respectively, increased and reduced relative to controls, proportional to the severity of each thalassemia group. In conclusion, we showed in this study, for the first time in the literature, that thalassemia carriers have altered iron metabolism and erythropoiesis.

KEYWORDS:

GDF15; erythropoiesis; erythropoietin; ferritin; hepcidin; iron metabolism; soluble transferrin receptor; thalassemia

PMID:
25307880
PMCID:
PMC4393762
DOI:
10.1111/ejh.12464
[Indexed for MEDLINE]
Free PMC Article

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