Evaluation of cardiac iron overload in transfusion-dependent adult marrow failure patients by magnetic resonance imaging

Abstract Background Thalassemia is the most prevalent single-gene disorder. Myocardial and hepatic iron depositions lead to complications and eventually death. We aimed to assess the diagnostic efficacy of magnetic resonance imaging T2* (MRI T2*) in quantifying iron overload in liver and heart in transfusion-dependent B-thalassemia major (TDT) children. Methods Prospective clinical study was carried on sixty children diagnosed with TDT. All of them underwent laboratory investigations, including CBC, serum iron, and ferritin levels. MRI T2* of the heart and liver was carried out to measure the iron overload and estimate the left ventricular ejection fraction (LVEF). Results Thirty-eight males and 22 females with TDT with a mean age of 13.23 years were included. Twenty cases (33.3%) had severe liver iron overload, while 36 (60%) had normal cardiac iron. There was a moderate significant negative association between hepatic and cardiac iron deposition (P = 0.03). All cases with severe cardiac iron overload had impaired LVEF below 56%. A non-significant positive association was noticed between cardiac iron deposition and LVEF in T2* (P = 0.08). A moderate negative significant association was detected between hepatic iron deposition and serum ferritin, while a fair negative significant association was found between serum ferritin and cardiac iron deposition with P values of 0.04 and 0.02, respectively. Conclusion MRI T2* is the gold standard for monitoring and follow-up of iron overload in the heart and liver. It should be routinely performed in all TDT children as liver iron, and serum ferritin do not reflect cardiac iron overload.

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Low Serum Ferritin Levels are Misleading for Detecting Cardiac Iron Overload and Increase the Risk of Cardiomyopathy in Thalassemia Patients. The Importance of Cardiac Iron Overload Monitoring Using Magnetic Resonance Imaging T2 and T2*

Hemoglobin ◽

10.1080/03630260600642542 ◽

2006 ◽

Vol 30 (2) ◽

pp. 219-227 ◽

Cited By ~ 49

Author(s):

Annita Kolnagou ◽

Charalambos Economides ◽

Eleni Eracleous ◽

George J. Kontoghiorghes

Keyword(s):

Magnetic Resonance Imaging ◽

Magnetic Resonance ◽

Iron Overload ◽

Serum Ferritin ◽

Resonance Imaging ◽

Cardiac Iron ◽

Cardiac Iron Overload ◽

Low Serum

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Stratification for Age in Non-Transfusion-Dependent Thalassemia: Data from a National Italian Networking

Blood ◽

10.1182/blood-2018-99-112044 ◽

2018 ◽

Vol 132 (Supplement 1) ◽

pp. 3639-3639

Author(s):

Alessia Pepe ◽

Laura Pistoia ◽

Nicola Romano ◽

Giuseppe Colaci ◽

Aurelio Maggio ◽

...

Keyword(s):

Magnetic Resonance Imaging ◽

Magnetic Resonance ◽

Iron Overload ◽

Pediatric Patients ◽

Cardiac Complications ◽

Hepatic Iron ◽

Resonance Imaging ◽

Liver Iron ◽

Cardiac Iron ◽

Magnetic Resonance Imaging Mri

Abstract Background: Non-transfusion-dependent thalassemia (NTDT) is a term used to indicate patients who do not require lifelong regular transfusions for survival. Morbidity in NTDT patients is more common and serious than previously recognized. This study aimed to examine the association of age with the presence of iron overload assessed by Magnetic Resonance Imaging (MRI) and cardiovascular and endocrine complications in NTDT patients. Methods: We considered 170 patients with thalassemia intermedia never transfused o who received occasional transfusions consecutively enrolled in the Myocardial Iron Overload in Thalassemia (MIOT) project. Iron overload was quantified by the T2* Magnetic Resonance Imaging (MRI) technique. All complications were classified according to international guidelines. Results: Six groups of patients were identified: age<18 years (N=7), age 18-30 years (N=36), age 30-40 years (N=48), age 40-50 (N=45), age 50-60 years (N=26), and age≥60 years (N=8). The Table shows the comparison among the groups. The youngest patient showing hepatic iron (MRI liver iron concentration-LIC>3 mg/g dw) had 9 years of age and the frequency of hepatic iron did not significantly increase with age. Only one patient showed cardiac iron (global heart T2*<20 ms). Diabetes appeared only in patients with more than 50 years and showed a trend toward increasing with increasing age. Hypothyroidism and osteoporosis were not present in pediatric patients and were not associated to age. Hypogonadism was not present in patients with less than 30 years and its frequency was comparable among the age groups. No patient showed hypoparathyroidism. Only patients older than 30 years showed a cardiac complication (heart failure or arrhythmias), but the rate did not significantly increase with increasing age. Conclusions: Our data in NTDT are indicative of high rate of liver iron overload at early age and extremely rare cardiac iron overload. Endocrine or cardiac complications were not present in pediatric patients but in adult patients the frequency did not increase with advancing age. Table. Table. Disclosures Pepe: Chiesi Farmaceutici S.p.A., ApoPharma Inc., and Bayer: Other: No profit support.

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Prevention of Cardiomyopathy in Transfusion-Dependent Homozygous Thalassaemia Today and the Role of Cardiac Magnetic Resonance Imaging

Advances in Hematology ◽

10.1155/2009/964897 ◽

2009 ◽

Vol 2009 ◽

pp. 1-4 ◽

Cited By ~ 2

Author(s):

Athanassios Aessopos ◽

Vasilios Berdoukas ◽

Maria Tsironi

Keyword(s):

Magnetic Resonance Imaging ◽

Cardiac Magnetic Resonance ◽

Magnetic Resonance ◽

Cardiac Magnetic Resonance Imaging ◽

Cardiac Disease ◽

Iron Status ◽

Iron Chelation ◽

Resonance Imaging ◽

Cardiac Iron ◽

Cardiac Iron Overload

Transfusion and iron chelation therapy revolutionised survival and reduced morbidity in patients with transfusion-dependent beta thalassaemia major. Despite these improvements, cardiac disease remained the most common cause of death in those patients. Recently the ability to determine the degree of cardiac iron overload, through cardiac magnetic resonance imaging (CMR) has allowed more logical approaches to iron removal, particularly from the heart. The availability of two oral chelators, deferiprone and deferasirox has reduced the need for the injectable chelator deferrioxamine and an additional benefit has been that deferiprone has been shown to be more cardioprotective than deferrioxamine. This review on the prevention of cardiac disease makes recommendations on the chelation regime that would be desirable for patients according to their cardiac iron status as determined by CMR determined by CMR. It also discusses approaches to chelation management should CMR not be available.

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