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

Author:

Jones Emma1,Pasricha Sant-Rayn1,Allen Angela2,Evans Patricia3,Fisher Chris A.1,Wray Katherine1,Premawardhena Anuja4,Bandara Dyananda5,Perera Ashok5,Webster Craig6,Sturges Pamela6,Olivieri Nancy F.7,St. Pierre Timothy8,Armitage Andrew E.1,Porter John B.3,Weatherall David J.1,Drakesmith Hal1

Affiliation:

1. Medical Research Council, Weatherall Institute of Molecular Medicine, University of Oxford, Oxford, United Kingdom;

2. College of Medicine, Swansea University, Swansea, United Kingdom;

3. University College of London Cancer Institute, Department of Haematology, University College London, United Kingdom;

4. University of Kelaniya, Colombo, Sri Lanka;

5. National Thalassaemia Centre, District Hospital, Kurunegala, Sri Lanka;

6. Department of Biochemistry and Immunology, Birmingham Heartlands Hospital, Birmingham, United Kingdom;

7. University Health Network, University of Toronto, ON, Canada; and

8. School of Physics, University of Western Australia, Crawley, Australia

Abstract

Key Points Expanded erythropoiesis strongly drives hepcidin suppression in severe transfusion-dependent HbE β-thalassemia. β-thalassemia carriers, but not HbE carriers, have enhanced erythropoiesis associated with mildly suppressed hepcidin.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

Reference42 articles.

1. Hemoglobin e syndromes.;Vichinsky;Hematology (Am Soc Hematol Educ Program),2007

2. The inherited diseases of hemoglobin are an emerging global health burden.;Weatherall;Blood,2010

3. Hemoglobin E thalassemia.;Fucharoen,2013

4. Haemoglobin E beta thalassaemia in Sri Lanka.;Premawardhena;Lancet,2005

5. Survival and complications in thalassemia.;Borgna-Pignatti;Ann N Y Acad Sci,2005

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