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Hepcidin is suppressed by erythropoiesis in hemoglobin E β-thalassemia and β-thalassemia trait.

Jones, Emma and Pasricha, Sant-Rayn and Allen, Angela and Evans, Patricia and Fisher, Chris A and Wray, Katherine and Premawardhena, Anuja and Bandara, Dyananda and Perera, Ashok and Webster, Craig and Sturges, Pamela and Olivieri, Nancy F and St Pierre, Timothy and Armitage, Andrew E and Porter, John B and Weatherall, David J and Drakesmith, Hal (2015) Hepcidin is suppressed by erythropoiesis in hemoglobin E β-thalassemia and β-thalassemia trait. Blood, 125 (5). pp. 873-80. ISSN 1528-0020.

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Official URL: http://www.bloodjournal.org/content/125/5/873?sso-...

Abstract

Hemoglobin E (HbE) β-thalassemia is the most common severe thalassemia syndrome across Asia, and millions of people are carriers. Clinical heterogeneity in HbE β-thalassemia is incompletely explained by genotype, and the interaction of phenotypic variation with hepcidin is unknown. The effect of thalassemia carriage on hepcidin is also unknown, but it could be relevant for iron supplementation programs aimed at combating anemia. In 62 of 69 Sri Lankan patients with HbE β-thalassemia with moderate or severe phenotype, hepcidin was suppressed, and overall hepcidin inversely correlated with iron accumulation. On segregating by phenotype, there were no differences in hepcidin, erythropoiesis, or hemoglobin between severe or moderate disease, but multiple linear regression showed that erythropoiesis inversely correlated with hepcidin only in severe phenotypes. In moderate disease, no independent predictors of hepcidin were identifiable; nevertheless, the low hepcidin levels indicate a significant risk for iron overload. In a population survey of Sri Lankan schoolchildren, β-thalassemia (but not HbE) trait was associated with increased erythropoiesis and mildly suppressed hepcidin, suggesting an enhanced propensity to accumulate iron. In summary, the influence of erythropoiesis on hepcidin suppression associates with phenotypic disease variation and pathogenesis in HbE β-thalassemia and indicates that the epidemiology of β-thalassemia trait requires consideration when planning public health iron interventions.

Item Type: Article
Subjects: WH Haemic and lymphatic systems. Haematology
Divisions: Planned IP Care > Oncology and Clinical Haematology
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Depositing User: Miss Adele Stanton
Date Deposited: 31 May 2015 06:23
Last Modified: 31 May 2015 06:23
URI: http://www.repository.heartofengland.nhs.uk/id/eprint/903

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