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.
Mariani, R., Trombini, P., Pozzi, M., & Piperno, A. (2009). Iron metabolism in thalassemia and sickle cell disease. MEDITERRANEAN JOURNAL OF HEMATOLOGY AND INFECTIOUS DISEASES, 1(1).
|Citazione:||Mariani, R., Trombini, P., Pozzi, M., & Piperno, A. (2009). Iron metabolism in thalassemia and sickle cell disease. MEDITERRANEAN JOURNAL OF HEMATOLOGY AND INFECTIOUS DISEASES, 1(1).|
|Tipo:||Articolo in rivista - Articolo scientifico|
|Carattere della pubblicazione:||Scientifica|
|Titolo:||Iron metabolism in thalassemia and sickle cell disease|
|Autori:||Mariani, R; Trombini, P; Pozzi, M; Piperno, A|
|Data di pubblicazione:||ott-2009|
|Rivista:||MEDITERRANEAN JOURNAL OF HEMATOLOGY AND INFECTIOUS DISEASES|
|Digital Object Identifier (DOI):||http://dx.doi.org/10.4084/MJHID.2009.006|
|Appare nelle tipologie:||01 - Articolo su rivista|