IRON METABOLISM IN THALASSEMIA AND SICKLE CELL DISEASE

Raffaella Mariani, Paola Trombini, Matteo Pozzi, Alberto Piperno
  • Raffaella Mariani
    Centre for Diagnosis and Treatment of Hemochromatosis S.Gerardo Hospital Monza, Italy
  • Paola Trombini
    Affiliation not present
  • Matteo Pozzi
    Affiliation not present

Abstract

There are two main mechanisms by which iron overload develops in thalassemias: increased iron absorption due to ineffective erythropoiesis and blood transfusions. In nontransfused patients with severe thalassemia, abnormal dietary iron absorption increases body iron burden between 2 and 5 g per year. If regular transfusions are required, this doubles the rate of iron accumulation leading to earlier massive iron overload and iron-related damage. Iron metabolism largely differs between thalassemias and sickle cell disease, but chronic transfusion therapy partially normalize many of the disparities between the diseases, making iron overload an important issue to be considered in the management of patients with sickle cell disease too. The present review summarizes the actual knowledge on the regulatory pathways of iron homeostasis. In particular, the data presented indicate the inextricably link between erythropoiesis and iron metabolism and the key role of hepcidin in coordinating iron procurement according to erythropoietic requirement. The role of erythropoietin, hypoxia, erythroid-dependent soluble factors and iron in regulating hepcidin transcription are discussed as well as differences and similarities in iron homeostasis between thalassemia syndromes and sickle cell disease.

Keywords

Thalassemic Syndromes, Iron metabolism

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Submitted: 2014-06-11 11:31:14
Published: 2009-10-27 00:00:00
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