In:
American Journal of Hematology, Wiley, Vol. 90, No. 11 ( 2015-11), p. 1008-1012
Kurzfassung:
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. Am. J. Hematol. 90:1008–1012, 2015. © 2015 Wiley Periodicals, Inc.
Materialart:
Online-Ressource
ISSN:
0361-8609
,
1096-8652
Sprache:
Englisch
Verlag:
Wiley
Publikationsdatum:
2015
ZDB Id:
1492749-4