Differences in the erythropoiesis-hepcidin-iron store axis between hemoglobin H disease and -thalassemia intermedia

Differences in the erythropoiesis-hepcidin-iron store axis between hemoglobin H disease and β-thalassemia intermedia.

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

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 ...

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

Medical Research Council, Weatherall Institute of Molecular Medicine, University of Oxford, Oxford, United Kingdom; College of Medicine, Swansea University, Swansea, United Kingdom; University College of London Cancer Institute, Department of Haematology, University College London, United Kingdom; University of Kelaniya, Colombo, Sri Lanka; National Thalassaemia Centre, District Hospital, Kurun...

Serum erythropoietin and erythropoiesis in high- and low-fetal hemoglobin beta-thalassemia intermedia patients.

Clinical data suggest that in beta-thalassemia-intermedia patients, higher levels of circulating fetal hemoglobin (HbF) are associated with greater disease severity at comparable degrees of anemia. We assessed the influence of the amount of circulating HbF on serum erythropoietin (s-Epo) levels and on serum transferrin receptor, a measure of erythropoiesis, in 30 beta-thalassemia-intermedia pat...

Hepcidin and the iron-infection axis.

Iron lies at the center of a battle for nutritional resource between higher organisms and their microbial pathogens. The iron status of the human host affects the pathogenicity of numerous infections including malaria, HIV-1, and tuberculosis. Hepcidin, an antimicrobial-like peptide hormone, has emerged as the master regulator of iron metabolism. Hepcidin controls the absorption of dietary iron...