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Published in: La radiologia medica 8/2018

01-08-2018 | CARDIAC RADIOLOGY

Magnetic resonance imaging during management of patients with transfusion-dependent thalassemia: a single-center experience

Authors: Zeynep Karakas, Yasin Yilmaz, Zuhal Bayramoglu, Serap Karaman, Selime Aydogdu, Ayse Ozkan Karagenc, Deniz Tugcu, Memduh Dursun

Published in: La radiologia medica | Issue 8/2018

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Abstract

Background

Cardiac and hepatic magnetic resonance imaging evaluation during treatment can tailor physicians’ chelation therapy titrations.

Aim

The aim of the study was to assess the relationship of cardiac and hepatic T2* values with chelation therapy in patients with transfusion-dependent thalassemia (TDT).

Methods

A total of 106 patients with TDT who were followed up in Istanbul Medical Faculty Thalassemia Center were evaluated for the study. Forty-eight (45%) patients with TDT had more than one consecutive MRI examination. The patients were divided into three subgroups according to the cardiac T2* values as the high-risk group (T2* MRI < 10 ms), medium-risk group (T2* MRI 10-20 ms), and the low-risk group (T2* MRI > 20 ms).

Results

The majority of patients used DFX (deferasirox) (79%) and deferiprone (DFP) (17%). Approximately 80% of patients according to cardiac T2* value and 40% of patients according to hepatic T2* value were initially in the low-risk group. Patients with follow-up MRI examinations exhibited significant improvement in liver iron concentration, which correlated with an increase in hepatic T2* values. The decrease of liver iron concentration was prominent in the DFX group (p < 0.01). The serum ferritin level was significantly correlated with liver iron concentrations (rs = 0.65, p < 0.001), hepatic T2* value (rs = − 0.62, p < 0.001), but not with cardiac T2* value (rs = − 0.20, p = 0.07).

Conclusion

Cardiovascular and hepatic MRI is a useful follow-up tool during the assessment of risk groups and chelation therapy of patients with TDT. Consecutive MRI tests showed good monitoring of cardiac and liver iron overload.
Literature
1.
go back to reference Viprakasit V, Origa R (2014) Genetic basis, pathophysiology and diagnosis. In: Cappellini MD, Cohen A, Porter J, Taher A, Viprakasit V (eds). Guidelines for the management of transfusion-dependent thalassemia (TDT), 3rd edn. Nicosia:Thalassaemia International Federation (TIF) Publication, No. 20, pp 14–26 Viprakasit V, Origa R (2014) Genetic basis, pathophysiology and diagnosis. In: Cappellini MD, Cohen A, Porter J, Taher A, Viprakasit V (eds). Guidelines for the management of transfusion-dependent thalassemia (TDT), 3rd edn. Nicosia:Thalassaemia International Federation (TIF) Publication, No. 20, pp 14–26
2.
go back to reference Baksi AJ, Pennell DJ (2014) Randomized controlled trials of iron chelators for the treatment of cardiac siderosis in thalassaemia major. Front Pharmacol 5:217CrossRefPubMedPubMedCentral Baksi AJ, Pennell DJ (2014) Randomized controlled trials of iron chelators for the treatment of cardiac siderosis in thalassaemia major. Front Pharmacol 5:217CrossRefPubMedPubMedCentral
3.
go back to reference Borgna-Pignatti C, Cappellini MD, De Stefano P et al (2006) Cardiac morbidity and mortality in deferoxamine or deferiprone-treated patients with thalassemia major. Blood 107:3733–3737CrossRefPubMed Borgna-Pignatti C, Cappellini MD, De Stefano P et al (2006) Cardiac morbidity and mortality in deferoxamine or deferiprone-treated patients with thalassemia major. Blood 107:3733–3737CrossRefPubMed
4.
go back to reference Carpenter JP, Roughton M, Pennell DJ (2013) International survey of T2* cardiovascular magnetic resonance in beta-thalassemia major. Haematologica 98(9):1368–1374CrossRefPubMedPubMedCentral Carpenter JP, Roughton M, Pennell DJ (2013) International survey of T2* cardiovascular magnetic resonance in beta-thalassemia major. Haematologica 98(9):1368–1374CrossRefPubMedPubMedCentral
5.
go back to reference Wood JC (2014) Use of magnetic resonance imaging to monitor iron overload. Hematol Oncol Clin N Am 28(4):747–764CrossRef Wood JC (2014) Use of magnetic resonance imaging to monitor iron overload. Hematol Oncol Clin N Am 28(4):747–764CrossRef
6.
go back to reference Kirk P, Raughton M, Porter JB et al (2009) Cardiac T2* magnetic resonance for prediction of cardiac complications in thalassemia major. Circulation 120:1961–1968CrossRefPubMedPubMedCentral Kirk P, Raughton M, Porter JB et al (2009) Cardiac T2* magnetic resonance for prediction of cardiac complications in thalassemia major. Circulation 120:1961–1968CrossRefPubMedPubMedCentral
7.
go back to reference Auger D, Pennell DJ (2016) Cardiac complications in thalassemia major. Ann N Y AcadSci 1368(1):56–64CrossRef Auger D, Pennell DJ (2016) Cardiac complications in thalassemia major. Ann N Y AcadSci 1368(1):56–64CrossRef
8.
go back to reference Wood JC, Enriquez C, Ghugre N et al (2005) MRI R2 and R2* mapping accurately estimates hepatic iron concentration in transfusion-dependent thalassemia and sickle cell disease patients. Blood 106:1460–1465CrossRefPubMedPubMedCentral Wood JC, Enriquez C, Ghugre N et al (2005) MRI R2 and R2* mapping accurately estimates hepatic iron concentration in transfusion-dependent thalassemia and sickle cell disease patients. Blood 106:1460–1465CrossRefPubMedPubMedCentral
9.
go back to reference Wood JC (2011) Impact of iron assessment by MRI. Hematol Am Soc Hematol Educ Program 2011:443–450 Wood JC (2011) Impact of iron assessment by MRI. Hematol Am Soc Hematol Educ Program 2011:443–450
11.
go back to reference Deugnier Y, Turlin B, Ropert M et al (2011) Improvement in liver pathology of patients with β-thalassemia treated with deferasirox for at least 3 years. Gastroenterology 141(4):1202–1211CrossRefPubMed Deugnier Y, Turlin B, Ropert M et al (2011) Improvement in liver pathology of patients with β-thalassemia treated with deferasirox for at least 3 years. Gastroenterology 141(4):1202–1211CrossRefPubMed
12.
go back to reference Wahidiyat PA, Liauw F, Sekarsari D et al (2017) Evaluation of cardiac and hepatic iron overload in thalassemia major patients with T2* magnetic resonance imaging. Hematology 22(8):501–507PubMed Wahidiyat PA, Liauw F, Sekarsari D et al (2017) Evaluation of cardiac and hepatic iron overload in thalassemia major patients with T2* magnetic resonance imaging. Hematology 22(8):501–507PubMed
13.
go back to reference Pepe A, Meloni A, Capra M et al (2011) Deferasirox, deferiprone and desferrioxamine treatment in thalassemia major patients: cardiac iron and function comparison determined by quantitative magnetic resonance imaging. Haematologica 96(1):41–47CrossRefPubMed Pepe A, Meloni A, Capra M et al (2011) Deferasirox, deferiprone and desferrioxamine treatment in thalassemia major patients: cardiac iron and function comparison determined by quantitative magnetic resonance imaging. Haematologica 96(1):41–47CrossRefPubMed
14.
go back to reference Puliyel M, Sposto R, Berdoukas VA et al (2014) Ferritin trends do not predict changes in total body iron in patients with transfusional iron overload. Am J Hematol 89:391–394CrossRefPubMed Puliyel M, Sposto R, Berdoukas VA et al (2014) Ferritin trends do not predict changes in total body iron in patients with transfusional iron overload. Am J Hematol 89:391–394CrossRefPubMed
15.
go back to reference Westwood MA, Anderson LJ, Firmin DN et al (2003) Interscanner reproducibility of cardiovascular magnetic resonance T2* measurements of tissue iron in thalassemia. J MagnReson Imaging 18:616–620CrossRef Westwood MA, Anderson LJ, Firmin DN et al (2003) Interscanner reproducibility of cardiovascular magnetic resonance T2* measurements of tissue iron in thalassemia. J MagnReson Imaging 18:616–620CrossRef
16.
go back to reference Jensen PD, Jensen FT, Christensen T et al (2001) Indirect evidence for the potential ability of magnetic resonance imaging to evaluate the myocardial iron content in patients with transfusional iron overload. MAGMA 12(2–3):153–166CrossRefPubMed Jensen PD, Jensen FT, Christensen T et al (2001) Indirect evidence for the potential ability of magnetic resonance imaging to evaluate the myocardial iron content in patients with transfusional iron overload. MAGMA 12(2–3):153–166CrossRefPubMed
17.
go back to reference Chouliaras G, Berdoukas V, Ladis V et al (2001) Impact of magnetic resonance imaging on cardiac mortality in thalassemia major. J Magn Reson Imaging 34(1):56–59CrossRef Chouliaras G, Berdoukas V, Ladis V et al (2001) Impact of magnetic resonance imaging on cardiac mortality in thalassemia major. J Magn Reson Imaging 34(1):56–59CrossRef
18.
go back to reference Pepe A, Meloni A, Rossi G et al (2013) Cardiac and hepatic iron and ejection fraction in thalassemia major: multicentre prospective comparison of combined deferiprone and deferoxamine therapy against deferiprone or deferoxamine monotherapy. J Cardiovasc Magn Reson 15:1CrossRefPubMedPubMedCentral Pepe A, Meloni A, Rossi G et al (2013) Cardiac and hepatic iron and ejection fraction in thalassemia major: multicentre prospective comparison of combined deferiprone and deferoxamine therapy against deferiprone or deferoxamine monotherapy. J Cardiovasc Magn Reson 15:1CrossRefPubMedPubMedCentral
19.
go back to reference Fragasso A, Ciancio A, Mannarella C et al (2011) Myocardial iron overload assessed by magnetic resonance imaging (MRI)T2* in multi-transfused patients with thalassemia and acquired anemias. Eur J Intern Med 22(1):62–65CrossRefPubMed Fragasso A, Ciancio A, Mannarella C et al (2011) Myocardial iron overload assessed by magnetic resonance imaging (MRI)T2* in multi-transfused patients with thalassemia and acquired anemias. Eur J Intern Med 22(1):62–65CrossRefPubMed
20.
go back to reference Farhangi H, Badiei Z, Moghaddam HM et al (2017) Assessment of heart and liver iron overload in thalassemia major patients using T2* magnetic resonance imaging. Indian J Hematol Blood Transfus 33(2):228–234CrossRefPubMed Farhangi H, Badiei Z, Moghaddam HM et al (2017) Assessment of heart and liver iron overload in thalassemia major patients using T2* magnetic resonance imaging. Indian J Hematol Blood Transfus 33(2):228–234CrossRefPubMed
21.
go back to reference Akcay A, Salcioglu Z, Oztarhan K et al (2014) Cardiac T2* MRI assessment in patients with thalassaemia major and its effect on the preference of chelation therapy. Int J Hematol 99(6):706–713CrossRefPubMed Akcay A, Salcioglu Z, Oztarhan K et al (2014) Cardiac T2* MRI assessment in patients with thalassaemia major and its effect on the preference of chelation therapy. Int J Hematol 99(6):706–713CrossRefPubMed
22.
go back to reference Pennell DJ, Porter JB, Cappellini MD et al (2012) Deferasirox for up to 3 years leads to continued improvement of myocardial T2* in patients with β-thalassemia major. Haematologica 97(6):842–848CrossRefPubMedPubMedCentral Pennell DJ, Porter JB, Cappellini MD et al (2012) Deferasirox for up to 3 years leads to continued improvement of myocardial T2* in patients with β-thalassemia major. Haematologica 97(6):842–848CrossRefPubMedPubMedCentral
Metadata
Title
Magnetic resonance imaging during management of patients with transfusion-dependent thalassemia: a single-center experience
Authors
Zeynep Karakas
Yasin Yilmaz
Zuhal Bayramoglu
Serap Karaman
Selime Aydogdu
Ayse Ozkan Karagenc
Deniz Tugcu
Memduh Dursun
Publication date
01-08-2018
Publisher
Springer Milan
Published in
La radiologia medica / Issue 8/2018
Print ISSN: 0033-8362
Electronic ISSN: 1826-6983
DOI
https://doi.org/10.1007/s11547-018-0889-0

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