Extramedullary hematopoiesis is associated with lower cardiac iron loading in chronically transfused thalassemia patients. Issue 11 (8th October 2015)

• Record Type: Journal Article
• Authors: Ricchi, Paolo; Meloni, Antonella; Spasiano, Anna; Neri, Maria Giovanna; Gamberini, Maria Rita; Cuccia, Liana; Caruso, Vincenzo; Gerardi, Calogera; D'Ascola, Domenico Giuseppe; Rosso, Rosamaria; Campisi, Saveria; Rizzo, Michele; Terrazzino, Fabrizia; Vangosa, Alessandra Briatico; Chiodi, Elisabetta; Missere, Massimiliano; Mangione, Maurizio; Positano, Vincenzo; Pepe, Alessia
• Is Part Of: American journal of hematology. Volume 90:Issue 11(2015:Nov.)
• Page Start: 1008
• Page End: 1012
• Publication Date: 2015-10-08
• Subjects: Hematology -- Periodicals; 616.15
• DOI: 10.1002/ajh.24139
• Languages: English
• ISSNs: 0361-8609

Abstract

<abstract abstract-type="main"> <title> <x xml:space="preserve">Abstract</x> </title> <p>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 (<italic>P</italic> = 0.003). The patterns of cardiac iron distribution were significantly different in the EMH+ and EMH− patients (<italic>P</italic> < 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 (<italic>P</italic> = 0.001) and a significantly higher pulmonary artery diameter (<italic>P</italic> = 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. Am. J. Hematol. 90:1008–1012, 2015. © 2015 Wiley Periodicals, Inc.</p> </abstract>