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Thalassemia Reports
Volume 4
Issue 2
10.4081/thal.2014.2729
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Article

Role of Iron Metabolism Genetic Determinants in Response to Chelation Therapy in a Cohort of β-Thalassemia and Sickle Cell Syndromes Italian Patients

by
Maria Concetta Renda
*,
Disma Renda
,
Angela Piazza
,
Giuseppina Calvaruso
,
Emanuela Fecarotta
,
Antonino Giangreco
and
Aurelio Maggio
UOC Ematologia per le Malattie Rare del Sangue e degli Organi Ematopoietici, AO Ospedali Riuniti Villa Sofia-Cervello, 90146 Palermo, Italy
*
Author to whom correspondence should be addressed.
Thalass. Rep. 2014, 4(2), 2729; https://doi.org/10.4081/thal.2014.2729
Submission received: 18 February 2014 / Revised: 23 June 2014 / Accepted: 2 July 2014 / Published: 29 September 2014

Abstract

In patients with β-thalassemia and sickle cell syndromes there is an important secondary iron overload due to regular blood transfusions and increased duodenal iron absorption. As in genetic hemochromatosis, also the secondary iron storage leads to tissue injury that involves all the major organs: liver, heart, kidney, endocrine glands. At present, in patients with β-thalassemia and sickle cell syndrome, iron chelation therapy is widely used for the treatment of secondary hemochromatosis, to limit the toxic effects of iron overload. In order to maintain the correct homeostasis, several genes are involved in the metabolic pathways of iron, including HFE, FPN (ferroportin) and TF (transferrin). In this study we analyzed the genes HFE, FPN and TF, to assess their possible effects on response to therapy with deferasirox and deferiprone, either as monotherapy or in combination therapy in a cohort of patients with β-thalassemia and sickle cell syndromes.
Keywords: chelation therapy; iron overload; β-thalassemia; sickle cell syndromes chelation therapy; iron overload; β-thalassemia; sickle cell syndromes

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MDPI and ACS Style

Renda, M.C.; Renda, D.; Piazza, A.; Calvaruso, G.; Fecarotta, E.; Giangreco, A.; Maggio, A. Role of Iron Metabolism Genetic Determinants in Response to Chelation Therapy in a Cohort of β-Thalassemia and Sickle Cell Syndromes Italian Patients. Thalass. Rep. 2014, 4, 2729. https://doi.org/10.4081/thal.2014.2729

AMA Style

Renda MC, Renda D, Piazza A, Calvaruso G, Fecarotta E, Giangreco A, Maggio A. Role of Iron Metabolism Genetic Determinants in Response to Chelation Therapy in a Cohort of β-Thalassemia and Sickle Cell Syndromes Italian Patients. Thalassemia Reports. 2014; 4(2):2729. https://doi.org/10.4081/thal.2014.2729

Chicago/Turabian Style

Renda, Maria Concetta, Disma Renda, Angela Piazza, Giuseppina Calvaruso, Emanuela Fecarotta, Antonino Giangreco, and Aurelio Maggio. 2014. "Role of Iron Metabolism Genetic Determinants in Response to Chelation Therapy in a Cohort of β-Thalassemia and Sickle Cell Syndromes Italian Patients" Thalassemia Reports 4, no. 2: 2729. https://doi.org/10.4081/thal.2014.2729

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