Cardiac iron overload evaluation in thalassaemic patients using T2* magnetic resonance imaging following chelation therapy: a multicentre cross-sectional study
Hematol., Transfus. Cell Ther. (Impr.)
;
45(1): 7-15, Jan.-Mar. 2023. tab, graf
Article
in English
| LILACS
| ID: biblio-1421549
ABSTRACT
Abstract Introduction Magnetic resonance imaging (MRI) T2* technique is used to assess iron overload in the heart, liver and pancreas of thalassaemic patients. Optimal iron chelation and expected tissue iron response rates remain under investigation. The objective of this study was to analyse serum ferritin and the iron concentration in the heart, liver and pancreas measured by MRI T2*/R2* during regular chelation therapy in a real-world cohort of patients with thalassemia. Methods We evaluated thalassaemic patients ≥ 7 years old undergoing chelation/transfusion therapy by MRI and assessed serum ferritin at baseline and follow-up from 2004-2011. Results We evaluated 136 patients, 92% major thalassaemic, with a median age of 18 years, and median baseline ferritin 2.033ng/ml (range 59-14,123). Iron overload distribution was liver (99%), pancreas (74%) and heart (36%). After a median of 1.2 years of follow-up, the iron overload in the myocardium reduced from 2,63 Fe mg/g to 2,05 (p 0.003). The optimal R2* pancreas cut-off was 148 Hertz, achieving 78% sensitivity and 73% specificity. However, when combining the R2* pancreas cut off ≤ 50 Hertz and a ferritin ≤ 1222 ng/ml, we could reach a negative predictive value (NPV) of 98% for cardiac siderosis. Only 28% were undergoing combined chelation at baseline assessment, which increased up to 50% on follow up evaluation. Conclusions Chelation therapy significantly reduced cardiac siderosis in thalassaemic patients. In patients with moderate/severe liver iron concentration undergoing chelation therapy, ferritin levels and myocardium iron improved earlier than the liver siderosis.
Full text:
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Index:
LILACS (Americas)
Main subject:
Thalassemia
/
Iron Overload
Type of study:
Observational study
/
Prevalence study
/
Risk factors
Limits:
Child
/
Humans
Language:
English
Journal:
Hematol., Transfus. Cell Ther. (Impr.)
Journal subject:
Hematologia
/
TransfusÆo de Sangue
Year:
2023
Type:
Article
Affiliation country:
Brazil
/
Canada
Institution/Affiliation country:
Associação Brasileira de Linfoma e Leucemia/BR
/
Centro de Hematologia e Hemoterapia do Paraná/BR
/
Centro de Pesquisa Boldrini/BR
/
Clinical Trials Department Apotex/CA
/
Fundação de Hematologia e Hemoterapia de Pernambuco/BR
/
Hemocentro de Marília/BR
/
Hospital Infantil Sabará/BR
/
Hospital Israelita Albert Einstein/BR
/
Universidade de Campinas/BR
/
Universidade de São Paulo (USP)/BR
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