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
em En
| LILACS
| ID: biblio-1421549
Biblioteca responsável:
BR408.1
Localização: BR408.1
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.
Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
LILACS
Assunto principal:
Talassemia
/
Sobrecarga de Ferro
Tipo de estudo:
Observational_studies
/
Prevalence_studies
/
Risk_factors_studies
Limite:
Child
/
Humans
Idioma:
En
Revista:
Hematol Transfus Cell Ther
/
Hematol., Transfus. Cell Ther. (Impr.)
/
Hematology, Transfusion and Cell Therapy (Impresso)
/
Hematology, Transfusion and Cell Therapy (Online)
Assunto da revista:
Hematologia
/
TransfusÆo de Sangue
Ano de publicação:
2023
Tipo de documento:
Article
País de afiliação:
Brasil
/
Canadá
País de publicação:
Brasil