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.
journal_name
Bloodjournal_title
Bloodauthors
Jones E,Pasricha SR,Allen A,Evans P,Fisher CA,Wray K,Premawardhena A,Bandara D,Perera A,Webster C,Sturges P,Olivieri NF,St Pierre T,Armitage AE,Porter JB,Weatherall DJ,Drakesmith Hdoi
10.1182/blood-2014-10-606491subject
Has Abstractpub_date
2015-01-29 00:00:00pages
873-80issue
5eissn
0006-4971issn
1528-0020pii
blood-2014-10-606491journal_volume
125pub_type
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