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Cardiac iron overload evaluation in thalassaemic patients using T2* magnetic resonance imaging following chelation
Eduardo Cerello Chapchap1, Murilo Marques Almeida Silva1, Reijane Alves de Assis2
1Hospital Israelita Albert Einstein, São Paulo, SP, Brazil.
Insights
Chelation therapy effectively reduced cardiac iron overload in thalassemia patients. Monitoring serum ferritin and pancreas iron with MRI T2* can help predict cardiac siderosis risk.
Area of Science:
- Hematology
- Radiology
- Cardiology
Background:
- Thalassemia patients often develop iron overload, necessitating regular chelation therapy.
- Magnetic Resonance Imaging (MRI) T2* technique is crucial for assessing iron levels in organs like the heart, liver, and pancreas.
- Optimal chelation strategies and tissue iron response rates require ongoing investigation.
Purpose of the Study:
- To analyze serum ferritin and organ iron concentrations (heart, liver, pancreas) using MRI T2*/R2* in a real-world cohort of thalassemia patients undergoing chelation therapy.
Main Methods:
- 136 thalassemia patients (≥7 years) on chelation/transfusion therapy were evaluated using MRI and serum ferritin levels from 2004-2011.
- Baseline and follow-up assessments were conducted to track changes in iron levels.
Main Results:
- Chelation therapy significantly reduced myocardial iron overload (p=0.003) over a median follow-up of 1.2 years.
- An R2* pancreas cut-off of 148 Hertz showed 78% sensitivity and 73% specificity for pancreatic siderosis.
- Combining R2* pancreas ≤ 50 Hertz and ferritin ≤ 1222 ng/ml achieved a 98% negative predictive value for cardiac siderosis.
Conclusions:
- Chelation therapy demonstrated a significant reduction in cardiac siderosis among thalassemia patients.
- In patients with moderate to severe liver iron, serum ferritin and myocardial iron improved faster than liver siderosis during chelation.
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.
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