Literature DB >> 22271905

Deferasirox for up to 3 years leads to continued improvement of myocardial T2* in patients with β-thalassemia major.

Dudley J Pennell1, John B Porter, Maria Domenica Cappellini, Lee Lee Chan, Amal El-Beshlawy, Yesim Aydinok, Hishamshah Ibrahim, Chi-Kong Li, Vip Viprakasit, Mohsen S Elalfy, Antonis Kattamis, Gillian Smith, Dany Habr, Gabor Domokos, Bernard Roubert, Ali Taher.   

Abstract

BACKGROUND: Prospective data on cardiac iron removal are limited beyond one year and longer-term studies are, therefore, important. DESIGN AND METHODS: Seventy-one patients in the EPIC cardiac substudy elected to continue into the 3(rd) year, allowing cardiac iron removal to be analyzed over three years.
RESULTS: Mean deferasirox dose during year 3 was 33.6 ± 9.8 mg/kg per day. Myocardial T2*, assessed by cardiovascular magnetic resonance, significantly increased from 12.0 ms ± 39.1% at baseline to 17.1 ms ± 62.0% at end of study (P<0.001), corresponding to a decrease in cardiac iron concentration (based on ad hoc analysis of T2*) from 2.43 ± 1.2 mg Fe/g dry weight (dw) at baseline to 1.80 ± 1.4 mg Fe/g dw at end of study (P<0.001). After three years, 68.1% of patients with baseline T2* 10 to <20 ms normalized (≥ 20 ms) and 50.0% of patients with baseline T2* >5 to <10 ms improved to 10 to <20 ms. There was no significant variation in left ventricular ejection fraction over the three years. No deaths occurred and the most common investigator-assessed drug-related adverse event in year 3 was increased serum creatinine (n = 9, 12.7%).
CONCLUSIONS: Three years of deferasirox treatment along with a clinically manageable safety profile significantly reduced cardiac iron overload versus baseline and normalized T2* in 68.1% (32 of 47) of patients with T2* 10 to <20 ms.

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Year:  2012        PMID: 22271905      PMCID: PMC3366648          DOI: 10.3324/haematol.2011.049957

Source DB:  PubMed          Journal:  Haematologica        ISSN: 0390-6078            Impact factor:   9.941


  27 in total

1.  Relation of myocardial T2* to right ventricular function in thalassaemia major.

Authors:  Francisco Alpendurada; John-Paul Carpenter; Monica Deac; Paul Kirk; J Malcolm Walker; John B Porter; Winston Banya; Taigang He; Gill C Smith; Dudley J Pennell
Journal:  Eur Heart J       Date:  2010-04-22       Impact factor: 29.983

2.  Continued improvement in myocardial T2* over two years of deferasirox therapy in β-thalassemia major patients with cardiac iron overload.

Authors:  Dudley J Pennell; John B Porter; Maria Domenica Cappellini; Lee Lee Chan; Amal El-Beshlawy; Yesim Aydinok; Hishamshah Ibrahim; Chi-Kong Li; Vip Viprakasit; Mohsen Saleh Elalfy; Antonis Kattamis; Gillian Smith; Dany Habr; Gabor Domokos; Bernard Roubert; Ali Taher
Journal:  Haematologica       Date:  2010-11-11       Impact factor: 9.941

3.  Efficacy of deferasirox in reducing and preventing cardiac iron overload in beta-thalassemia.

Authors:  Dudley J Pennell; John B Porter; Maria Domenica Cappellini; Amal El-Beshlawy; Lee Lee Chan; Yesim Aydinok; Mohsen Saleh Elalfy; Pranee Sutcharitchan; Chi-Kong Li; Hishamshah Ibrahim; Vip Viprakasit; Antonis Kattamis; Gillian Smith; Dany Habr; Gabor Domokos; Bernard Roubert; Ali Taher
Journal:  Blood       Date:  2009-12-08       Impact factor: 22.113

4.  The effect of deferasirox on cardiac iron in thalassemia major: impact of total body iron stores.

Authors:  John C Wood; Barinder P Kang; Alexis Thompson; Patricia Giardina; Paul Harmatz; Tara Glynos; Carole Paley; Thomas D Coates
Journal:  Blood       Date:  2010-04-26       Impact factor: 22.113

5.  Deferasirox, deferiprone and desferrioxamine treatment in thalassemia major patients: cardiac iron and function comparison determined by quantitative magnetic resonance imaging.

Authors:  Alessia Pepe; Antonella Meloni; Marcello Capra; Paolo Cianciulli; Luciano Prossomariti; Cristina Malaventura; Maria Caterina Putti; Alma Lippi; Maria Antonietta Romeo; Maria Grazia Bisconte; Aldo Filosa; Vincenzo Caruso; Antonella Quarta; Lorella Pitrolo; Massimiliano Missere; Massimo Midiri; Giuseppe Rossi; Vincenzo Positano; Massimo Lombardi; Aurelio Maggio
Journal:  Haematologica       Date:  2010-09-30       Impact factor: 9.941

6.  Follow-up report on the 2-year cardiac data from a deferasirox monotherapy trial.

Authors:  John C Wood; Tara Glynos; Alexis Thompson; Patricia Giardina; Paul Harmatz; Barinder P Kang; Carole Paley; Thomas D Coates
Journal:  Am J Hematol       Date:  2010-10       Impact factor: 10.047

7.  Reduction of body iron stores to normal range levels in thalassaemia by using a deferiprone/deferoxamine combination and their maintenance thereafter by deferiprone monotherapy.

Authors:  Annita Kolnagou; Marios Kleanthous; George J Kontoghiorghes
Journal:  Eur J Haematol       Date:  2010-11       Impact factor: 2.997

8.  Relation of chelation regimes to cardiac mortality and morbidity in patients with thalassaemia major: an observational study from a large Greek Unit.

Authors:  Vassilios Ladis; Giorgos Chouliaras; Vasilios Berdoukas; Panagiotis Moraitis; Kirykos Zannikos; Eleni Berdoussi; Christos Kattamis
Journal:  Eur J Haematol       Date:  2010-08-30       Impact factor: 2.997

9.  Cell functions impaired by frataxin deficiency are restored by drug-mediated iron relocation.

Authors:  Or Kakhlon; Hila Manning; William Breuer; Naomi Melamed-Book; Chunye Lu; Gino Cortopassi; Arnold Munnich; Z Ioav Cabantchik
Journal:  Blood       Date:  2008-09-16       Impact factor: 22.113

10.  Combined chelation therapy in thalassemia major for the treatment of severe myocardial siderosis with left ventricular dysfunction.

Authors:  Mark A Tanner; Renzo Galanello; Carlo Dessi; Gillian C Smith; Mark A Westwood; Annalisa Agus; Martina Pibiri; Sunil V Nair; J Malcolm Walker; Dudley J Pennell
Journal:  J Cardiovasc Magn Reson       Date:  2008-02-25       Impact factor: 5.364

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  37 in total

1.  Effects of deferasirox-deferoxamine on myocardial and liver iron in patients with severe transfusional iron overload.

Authors:  Yesim Aydinok; Antonis Kattamis; M Domenica Cappellini; Amal El-Beshlawy; Raffaella Origa; Mohsen Elalfy; Yurdanur Kilinç; Silverio Perrotta; Zeynep Karakas; Vip Viprakasit; Dany Habr; Niculae Constantinovici; Junwu Shen; John B Porter
Journal:  Blood       Date:  2015-05-01       Impact factor: 22.113

Review 2.  Deferasirox for managing iron overload in people with thalassaemia.

Authors:  Claudia Bollig; Lisa K Schell; Gerta Rücker; Roman Allert; Edith Motschall; Charlotte M Niemeyer; Dirk Bassler; Joerg J Meerpohl
Journal:  Cochrane Database Syst Rev       Date:  2017-08-15

3.  Risk factors for heart disease in transfusion-dependent thalassemia: serum ferritin revisited.

Authors:  Giorgio Derchi; Carlo Dessì; Patrizio Bina; Maria Domenica Cappellini; Antonio Piga; Silverio Perrotta; Immacolata Tartaglione; Marianna Giuditta; Filomena Longo; Raffaella Origa; Antonella Quarta; Valeria Pinto; Gian Luca Forni
Journal:  Intern Emerg Med       Date:  2018-06-12       Impact factor: 3.397

Review 4.  Impact of cardiac magnetic resonance imaging in non-ischemic cardiomyopathies.

Authors:  Kevin Kalisz; Prabhakar Rajiah
Journal:  World J Cardiol       Date:  2016-02-26

Review 5.  Iron overload in thalassemia: different organs at different rates.

Authors:  Ali T Taher; Antoine N Saliba
Journal:  Hematology Am Soc Hematol Educ Program       Date:  2017-12-08

Review 6.  Deferasirox nephrotoxicity-the knowns and unknowns.

Authors:  Juan Daniel Díaz-García; Angel Gallegos-Villalobos; Liliana Gonzalez-Espinoza; Maria D Sanchez-Niño; Jesus Villarrubia; Alberto Ortiz
Journal:  Nat Rev Nephrol       Date:  2014-07-22       Impact factor: 28.314

7.  Cardiac T2* MRI assessment in patients with thalassaemia major and its effect on the preference of chelation therapy.

Authors:  Arzu Akcay; Zafer Salcioglu; Kazim Oztarhan; Deniz Tugcu; Gonul Aydogan; Nuray Aktay Ayaz; Helen Bornaun; Hulya Sayilan Sen; Ferhan Akici; Burhan Akdana
Journal:  Int J Hematol       Date:  2014-04-10       Impact factor: 2.490

8.  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
Journal:  Radiol Med       Date:  2018-04-16       Impact factor: 3.469

Review 9.  Cardiac complications in beta-thalassemia: From mice to men.

Authors:  Sirinart Kumfu; Suthat Fucharoen; Siriporn C Chattipakorn; Nipon Chattipakorn
Journal:  Exp Biol Med (Maywood)       Date:  2017-05-09

10.  Combined chelation therapy with deferasirox and deferoxamine in thalassemia.

Authors:  Ashutosh Lal; John Porter; Nancy Sweeters; Vivian Ng; Patricia Evans; Lynne Neumayr; Gregory Kurio; Paul Harmatz; Elliott Vichinsky
Journal:  Blood Cells Mol Dis       Date:  2012-11-11       Impact factor: 3.039

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