title: Extramedullary hematopoiesis is associated with lower cardiac iron loading in chronically transfused thalassemia patients.
abstract: The aim of this study was to evaluate, in a large cohort of chronically transfused patients, whether the presence of extramedullary hematopoiesis (EMH) accounts for the typical patterns of cardiac iron distribution and/or cardiac function parameters. We retrospectively selected 1,266 thalassemia major patients who had undergone regular transfusions (611 men and 655 women; mean age: 31.3 ± 8.9 years, range: 4.2-66.6 years) and were consecutively enrolled within the Myocardial Iron Overload in Thalassemia network. The presence of EMH was evaluated based on steady-state free precession sequences; cardiac and liver iron overloads were quantified using a multiecho T2* approach; cardiac function parameters and pulmonary diameter were quantified using the steady-state free precession sequences; and myocardial fibrosis was evaluated using the late gadolinium enhancement technique. EMH was detected in 167 (13.2%) patients. The EMH+ patients had significantly lower cardiac iron overload than that of the EMH- patients (P = 0.003). The patterns of cardiac iron distribution were significantly different in the EMH+ and EMH- patients (P < 0.0001), with a higher prevalence of patients with no myocardial iron overload and heterogeneous myocardial iron overload and no significant global heart iron in the EMH+ group EMH+ patients had a significantly higher left ventricle mass index (P = 0.001) and a significantly higher pulmonary artery diameter (P = 0.002). In conclusion, in regularly transfused thalassemia patients, EMH was common and was associated with a thalassemia intermedia-like pattern of cardiac iron deposition despite regular transfusion therapy.
authors.affiliation: Unità Operativa Semplice Dipartimentale Malattie Rare Del Globulo Rosso, Azienda Ospedaliera Di Rilievo Nazionale "a. Cardarelli,", Napoli, Italy.
authors.forename: Paolo
authors.initials: P
authors.lastname: Ricchi
authors.affiliation: CMR Unit, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy.|U.O.C. Bioingegneria E Ingegneria Clinica, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy.
authors.forename: Antonella
authors.initials: A
authors.lastname: Meloni
authors.affiliation: Unità Operativa Semplice Dipartimentale Malattie Rare Del Globulo Rosso, Azienda Ospedaliera Di Rilievo Nazionale "a. Cardarelli,", Napoli, Italy.
authors.forename: Anna
authors.initials: A
authors.lastname: Spasiano
authors.affiliation: CMR Unit, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy.
authors.forename: Maria Giovanna
authors.initials: MG
authors.lastname: Neri
authors.affiliation: Dipartimento Della Riproduzione E Dell'accrescimento, Day Hospital Della Talassemia E Delle Emoglobinopatie, Azienda Ospedaliero-Universitaria Arcispedale "S. Anna,", Ferrara, Italy.
authors.forename: Maria Rita
authors.initials: MR
authors.lastname: Gamberini
authors.affiliation: Unità Operativa Complessa Ematologia Con Talassemia, ARNAS Civico "Benfratelli-Di Cristina,", Palermo, Italy.
authors.forename: Liana
authors.initials: L
authors.lastname: Cuccia
authors.affiliation: Unità Operativa Dipartimentale Talassemia, Presidio Ospedaliero Garibaldi-Centro - ARNAS Garibaldi, Catania, Italy.
authors.forename: Vincenzo
authors.initials: V
authors.lastname: Caruso
authors.affiliation: Presidio Ospedaliero "Giovanni Paolo II,", Distretto AG2 Di Sciacca, Sciacca (AG), Italy.
authors.forename: Calogera
authors.initials: C
authors.lastname: Gerardi
authors.affiliation: Centro Microcitemie, Azienda Ospedaliera "Bianchi-Melacrino-Morelli,", Reggio Calabria, Italy.
authors.forename: Domenico Giuseppe
authors.initials: DG
authors.lastname: D'Ascola
authors.affiliation: Unità Operativa Talassemie Ed Emoglobinopatie, Ospedale "Ferrarotto" - Azienda Ospedaliero-Universitaria Policlinico "Vittorio Emanuele,", Catania, Italy.
authors.forename: Rosamaria
authors.initials: R
authors.lastname: Rosso
authors.affiliation: Unità Operativa Semplice Talassemia, Presidio Ospedaliero "Umberto I,", Siracusa, Italy.
authors.forename: Saveria
authors.initials: S
authors.lastname: Campisi
authors.affiliation: Reparto Di Ematologia, Ospedale "Sant'elia,", altanisetta, Italy.
authors.forename: Michele
authors.initials: M
authors.lastname: Rizzo
authors.affiliation: Unità Operativa Complessa Radiologia, "ARNAS" Civico, Palermo, Italy.
authors.forename: Fabrizia
authors.initials: F
authors.lastname: Terrazzino
authors.affiliation: Institute of Radiology, Department of Neuroscience and Imaging, Section of Diagnostic Imaging and Therapy-Radiology Division, "G. D'Annunzio" University, Chieti, Italy.
authors.forename: Alessandra Briatico
authors.initials: AB
authors.lastname: Vangosa
authors.affiliation: Dipartimento Di Diagnostica per Immagini E Medicina Di Laboratorio, Struttura Complessa Di Radiologia Diagnostica E Interventistica Interaziendale, Azienda Ospedaliero-Universitaria Arcispedale "S. Anna-Cona,", Cona (FE), Italy.
authors.forename: Elisabetta
authors.initials: E
authors.lastname: Chiodi
authors.affiliation: Dipartimento Di Immagini, Fondazione Di Ricerca E Cura "Giovanni Paolo II,", Campobasso, Italy.
authors.forename: Massimiliano
authors.initials: M
authors.lastname: Missere
authors.affiliation: U.O.S. Sistemi Informatici, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy.
authors.forename: Maurizio
authors.initials: M
authors.lastname: Mangione
authors.affiliation: CMR Unit, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy.|U.O.C. Bioingegneria E Ingegneria Clinica, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy.
authors.forename: Vincenzo
authors.initials: V
authors.lastname: Positano
authors.affiliation: CMR Unit, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy.
authors.forename: Alessia
authors.initials: A
authors.lastname: Pepe
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D005260:Female; D006352:Heart Ventricles / Q000378:metabolism / Q000473:pathology; D006411:Hematopoiesis, Extramedullary; D006801:Humans; D007501:Iron / Q000378:metabolism*; D019190:Iron Overload / Q000209:etiology / Q000378:metabolism* / Q000473:pathology; D008099:Liver / Q000378:metabolism / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009206:Myocardium / Q000378:metabolism* / Q000473:pathology; D012189:Retrospective Studies; D065227:Transfusion Reaction; D017086:beta-Thalassemia / Q000378:metabolism* / Q000473:pathology / Q000628:therapy
chemical_list: D007501:Iron