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Cardiac iron removal and functional cardiac improvement by different iron chelation regimens in thalassemia major patients

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Abstract

Heart failure due to myocardial iron overload remains the leading cause of morbidity and mortality in adult thalassemia major (TM) patients. We evaluated the removal of cardiac iron and the changes of cardiac function by different iron chelation in TM patients by T2* cardiac magnetic resonance (CMR). Sixty-seven TM patients (27 males/40 females; mean age, 35 ± 6 years) on different chelation regimens underwent T2* CMR at baseline (t 0), after 6–14 months (t 1) and after 32 ± 7 months (t 2). Patients were divided in four groups according to chelation treatment: group A (deferasirox), group B (deferoxamine), group C (combined treatment, deferoxamine plus deferiprone) and group D (deferiprone alone). Myocardial T2* at t 0 was <10 ms in 8 patients, between 10 and 20 ms in 22 patients and ≥20 ms in 37 patients. Progressive changes in T2* were observed at t 1 and t 2. Ten patients (10/36, 27.8 %) in group A, three patients (3/15, 20 %) in group B and three patients (3/12, 25 %) in group C moved from an abnormal T2* to normal values. We observed an improvement of left ventricular ejection fraction and a reduction of end-systolic and end-diastolic left ventricular volumes only in patients in group A with baseline cardiac T2* between 10 and 20 ms. Rigorous compliance to any chelation therapy at proper doses significantly improve myocardial T2*. Treatment with deferasirox significantly improves left ventricular function. Combination therapy seems to ameliorate cardiac T2* in a shorter period of time in severe siderosis.

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References

  1. Borgna-Pignatti C, Rugolotto S, De Stefano P, Zhao H, Cappellini MD, Del Vecchio GC, Romeo MA, Forni GL, Gamberini MR, Ghilardi R, Piga A, Cnaan A (2004) Survival and complications in patients with thalassemia major treated with transfusion and deferoxamine. Haematologica 89:1187–1193

    PubMed  Google Scholar 

  2. Brittenham GM, Griffith PM, Nienhuis AW, McLaren CE, Young NS, Tuker EE, Allen CJ, Farrell DE, Harris JW (1994) Efficacy of deferoxamine in preventing complications of iron overload in patients with thalassemia major. N Engl J Med 331:567–573

    Article  PubMed  CAS  Google Scholar 

  3. Cappellini MD, Musallam KM, Taher AT (2009) Overview of iron chelation therapy with deferoxamine and deferiprone. Hemoglobin 33(suppl1):S58–69

    Article  PubMed  CAS  Google Scholar 

  4. Maggio A, Vitrano A, Capra M, Cuccia L, Gagliardetto F, Filosa A, Magnano C, Caruso V, Gerardi C, Argento C, Campisi S, Cantella F, Commendatore F, D’Ascola DG, Fidara C, Ciancio A, Galati MC, Giuffrida G, Cingari R, Giugno G, Lombardo T, Prossamariti I, Malizia R, Meo A, Roccamo G, Romeo MA, Violi P, Cianciulli P, Rigano P (2009) Improving survival with deferiprone treatment in patients with thalassemia major: a prospective, multicenter, randomised clinical trial under the auspices of the Italian Society for Thalassemia and Hemoglobinopathies. Blood Cell Mol Dis 42(3):247–251

    Article  CAS  Google Scholar 

  5. Pontikoglou C, Papadaki HA (2010) Idiosyncratic drug-induced agranulocytosis: the paradigm of deferiprone. Hemoglobin 34(3):291–304

    Article  PubMed  CAS  Google Scholar 

  6. Cappellini MD, Cohen A, Piga A, Bejaoui M, Perrotta S, Agaoglu L, Aydinok Y, Kattamis A, Kilinc Y, Porter J, Galanello R, Fattoum S, Drelichman G, Magnano C, Verissimo M, Athanassiou-Metaxa M, Giardina P, Kourakli-Symeonidis A, Janka-Schaub G, Coates T, Vermylen C, Olivieri N, Thuret I, Opitz H, Ressayre-Djaffer C, Marks P, Alberti D (2006) A phase 3 study of deferasirox (ICL670), a once-daily oral iron chelator in patients with β-thalassemia. Blood 107(9):3455–3462

    Article  PubMed  CAS  Google Scholar 

  7. Pennell D, Porter JB, Cappellini MD, El-Beshlawy A, Chan LL, Aydinok Y, Elalfy MS, Sutcharitchan P, Li CK, Ibrahim H, Viprakasit V, Kattamis A, Smith G, Habr D, Domokos G, Roubert B, Taher A (2008) Efficacy of deferasirox in reducing and preventing cardiac iron overload in β-thalassemia. Blood 115(12):2364–71

    Article  Google Scholar 

  8. Pennell D, Porter JB, Cappellini MD, Chan LL, El-Beshlawy A, Aydinok Y, Ibrahim H, Li CK, Viprakasit V, Elalfy MS, Kattamis A, Smith G, Habr D, Domokos G, Roubert B, Taher A (2011) Continued improvement in myocardial T2* over two years of deferasirox therapy in β-thalassemia major patients with cardiac iron overload. Hematologica 96(1):48–54

    Article  CAS  Google Scholar 

  9. Wood JC (2009) History and current impact of cardiac magnetic resonance imaging on the management of iron overload. Circulation 120:1937–1939

    Article  PubMed  Google Scholar 

  10. Westwood MA, Firmin DN, Gildo M, Renzo G, Stathis G, Markissia K, Vasili B, Pennel DJ (2005) Intercentre reproducibility of magnetic resonance T2* measurements of myocardial iron in thalassaemia. Int J Cardiovasc Imaging 21:531–538

    Article  PubMed  Google Scholar 

  11. Kirk P, Roughton M, Porter JB, Walker JM, Tanner MA, Patel J, Wu D, Taylor J, Westwood MA, Andreson LJ, Pennel DJ (2009) Cardiac T2* magnetic resonance for prediction of cardiac complications in thalassemia major. Circulation 120:1961–1968

    Article  PubMed  CAS  Google Scholar 

  12. Pennell DJ, Berdoukas V, Karagiorga M, Ladis V, Piga A, Aessopos A, Gotsis ED, Tanner MA, Smith GC, Westwood MA, Wonke B, Galanello R (2006) Randomized controlled trial of deferiprone or deferoxamine in beta-thalassemia major patients with asymptomatic myocardial siderosis. Blood 107:3738–3744

    Article  PubMed  CAS  Google Scholar 

  13. Wood JC, Kang BP, Thompson A, Giardina P, Harmatz P, Glynos T, Paley C, Coates TD (2010) The effect of deferasirox on cardiac iron in thalassemia major: impact of total body iron stores. Blood 116(4):537–543

    Article  PubMed  CAS  Google Scholar 

  14. Taher A, El-Beshlawy A, Elalfy MS, Alzir K, Daar S, Habr D, Krieulier-Krahn U, Hmissi A, Al Jefri A (2009) Efficacy and safety of deferasirox, an oral iron chelator, in heavily iron-overloaded patients with β-thalassaemia: the ESCALATOR study. Eur J Haematol 82(6):458–465

    Article  PubMed  CAS  Google Scholar 

  15. Pepe A, Meloni A, Capra M, Cianciulli P, Prossomariti L, Malaventura C, Putti MC, Lippi A, Romeo MA, Bisconte MG, Filona A, Caruso V, Quarta A, Pitrolo L, Missere M, Midiri M, Rossi G, Positano V, Lombardi M, Maggio A (2011) Deferasirox, deferiprone and desferrioxamine treatment in thalassemia major patients: cardiac iron and function determined by quantitative magnetic resonance imaging. Heamatologica 96(1):41–47

    Article  CAS  Google Scholar 

  16. Thalassemia International Federation (2007) Guidelines for the clinical management of thalassaemia 2nd ed.

  17. Borgna-Pignatti C, Cappellini MD, De Stefano P, Del Vecchio GC, Forni GL, Gamberini MR, Ghilardi R, Piga A, Romeo MA, Zhao H, Cnaan A (2006) Cardiac morbidity and mortality in deferoxamine- or deferiprone-treated patients with thalassemia major. Blood 107(9):3733–3738

    Article  PubMed  CAS  Google Scholar 

  18. Derchi G, Formisano F, Balocco M, Galanello R, Bina P, Dessì C, Piga A, Donato G, Cappellini MD, Cassinerio E, Quarta G, Melpignano A, Forni GL (2011) Clinical management of cardiovascular complications in patients with thalassemia major: a large observational multicenter study. Eur J Echocard 12(3):242–246

    Article  Google Scholar 

  19. Davis BA, Porter JB (2000) Long-term outcome of continuous 24-hour deferoxamine infusion via indwelling intravenous catheters in high-risk beta-thalassemia. Blood 95:1229–1236

    PubMed  CAS  Google Scholar 

  20. Lai ME, Grady RW, Vacquer S, Pepe A, Carta MP, Bina P, Sau F, Cianciulli P, Maggio A, Galanello R, Farci P (2010) Increased survival and reversion of iron-induced cardiac disease in patients with thalassemia major receiving intensive combined chelation therapy as compared to desferoxamine alone. Blood Cell Mol Dis 45:136–139

    Article  CAS  Google Scholar 

  21. Aessopos A, Fragodimitri C, Karabatsos F et al (2007) Cardiac magnetic resonance imaging R2* assessment and analysis of historical parameters in patients with transfusion-dependent thalassemia. Haematologica 92(1):131–132

    Article  PubMed  CAS  Google Scholar 

  22. Chouliaras GL, Kattamis A, Berdoukas V, Gotsis ED, Mavrogeni S, Ladis V (2010) Cardiac magnetic resonance in transfusion dependent thalassemia: assessment of iron load and relationship to left ventricular ejection fraction. Br J Haematol 151:397–401

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Hereditary Anemia Centre, Department of Internal Medicine, “Ca’ Granda” Foundation Ospedale Maggiore Policlinico IRCCS, University of Milan, Milan, Italy

    Elena Cassinerio, Francesca Brevi, Laura Zanaboni, Giovanna Graziadei, Paolo Pattoneri & Maria Domenica Cappellini

  2. CMR Unit, Department of Cardiology, Niguarda Ca’ Granda Hospital, Milan, Italy

    Alberto Roghi, Patrizia Pedrotti & Angela Milazzo

  3. Hereditary Anemia Center -Pad. Granelli- Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Via F. Sforza n.35, 20122, Milan, Italy

    Maria Domenica Cappellini

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  1. Elena Cassinerio
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  2. Alberto Roghi
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  3. Patrizia Pedrotti
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  4. Francesca Brevi
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  5. Laura Zanaboni
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  7. Paolo Pattoneri
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  8. Angela Milazzo
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  9. Maria Domenica Cappellini
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Correspondence to Maria Domenica Cappellini.

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Cassinerio, E., Roghi, A., Pedrotti, P. et al. Cardiac iron removal and functional cardiac improvement by different iron chelation regimens in thalassemia major patients. Ann Hematol 91, 1443–1449 (2012). https://doi.org/10.1007/s00277-012-1480-8

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  • DOI: https://doi.org/10.1007/s00277-012-1480-8

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