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

European Journal of Haematology

Volumn 85, Issue 4, 2010, Pages 335-344

  Ladis, Vassilios ^a   Chouliaras, Giorgos ^a   Berdoukas, Vasilios ^a   Moraitis, Panagiotis ^a   Zannikos, Kirykos ^a   Berdoussi, Eleni ^a   Kattamis, Christos ^a  

^a UNIVERSITY OF ATHENS   (Greece)

Author keywords

cardiac disease; deferasirox; deferiprone; desferrioxamine; thalassaemia major; transfusional iron overload

Indexed keywords

DEFERASIROX; DEFERIPRONE; DEFEROXAMINE; FERRITIN; HEMOGLOBIN; IRON; BENZOIC ACID DERIVATIVE; IRON CHELATING AGENT; PYRIDONE DERIVATIVE; TRIAZOLE DERIVATIVE;

ADULT; ARTICLE; BLOOD TRANSFUSION; CARDIOVASCULAR RISK; CHELATION THERAPY; FEMALE; FERRITIN BLOOD LEVEL; GREECE; HEART DEATH; HEART DISEASE; HEART FAILURE; HUMAN; IRON BLOOD LEVEL; IRON OVERLOAD; MAJOR CLINICAL STUDY; MALE; MORBIDITY; MORTALITY; NUCLEAR MAGNETIC RESONANCE; OBSERVATIONAL STUDY; PRIORITY JOURNAL; THALASSEMIA MAJOR; ADOLESCENT; AGE; BETA-THALASSEMIA; BLOOD; CHILD; COMPARATIVE STUDY; COMPLICATION; DEATH; HEART DISEASES; NUCLEAR MAGNETIC RESONANCE SPECTROSCOPY; PATHOPHYSIOLOGY; TREATMENT OUTCOME; YOUNG ADULT;

ADOLESCENT; ADULT; AGE FACTORS; BENZOATES; BETA-THALASSEMIA; BLOOD TRANSFUSION; CHILD; DEATH; DEFEROXAMINE; FEMALE; GREECE; HEART DISEASES; HUMANS; IRON; IRON CHELATING AGENTS; IRON OVERLOAD; MAGNETIC RESONANCE SPECTROSCOPY; MALE; PYRIDONES; TREATMENT OUTCOME; TRIAZOLES; YOUNG ADULT;

EID: 77956957627     PISSN: 09024441     EISSN: 16000609     Source Type: Journal    
DOI: 10.1111/j.1600-0609.2010.01491.x     Document Type: Article

References (30)

1. Modell B, Khan M, Darlison M. Survival in beta-thalassaemia major in the UK: data from the UK Thalassaemia Register. Lancet 2000 355 : 2051 2052.
2. Borgna-Pignatti C, Rugolotto S, De Stefano P, et al. Survival and complications in patients with thalassemia major treated with transfusion and deferoxamine. Haematologica 2004 89 : 1187 1193.
3. Ladis V, Chouliaras G, Berdousi H, Kanavakis E, Kattamis C. Longitudinal study of survival and causes of death in patients with thalassemia major in Greece. Ann N Y Acad Sci 2005 1054 : 445 450.
4. Chouliaras G, Yiannoutsos CT, Berdoukas V, Ladis V. Cardiac related death in thalassaemia major: time trend and risk factors in a large Greek Unit. Eur J Haematol 2009 82 : 381 387.
5. Caro JJ, Ward A, Green TC, et al. Impact of thalassemia major on patients and their families. Acta Haematol 2002 107 : 150 157.
6. Olivieri NF, Nathan DG, MacMillan JH, et al. Survival in medically treated patients with homozygous beta-thalassemia. N Engl J Med 1994 331 : 574 578.
7. Aessopos A, Farmakis D, Hatziliami A, et al. Cardiac status in well-treated patients with thalassemia major. Eur J Haematol 2004 73 : 359 366.
8. Modell B, Khan M, Darlison M, Westwood MA, Ingram D, Pennell DJ. Improved survival of thalassaemia major in the UK and relation to T2* cardiovascular magnetic resonance. J Cardiovasc Magn Reson 2008 10 : 42.
9. Telfer P, Coen PG, Christou S, et al. Survival of medically treated thalassemia patients in Cyprus. Trends and risk factors over the period 1980-2004. Haematologica 2006 91 : 1187 1192.
10. Piga A, Gaglioti C, Fogliacco E, Tricta F. Comparative effects of deferiprone and deferoxamine on survival and cardiac disease in patients with thalassemia major: a retrospective analysis. Haematologica 2003 88 : 489 496.
11. Borgna-Pignatti C, Cappellini MD, De Stefano P, et al. Cardiac morbidity and mortality in deferoxamine- or deferiprone-treated patients with thalassemia major. Blood 2006 107 : 3733 3737.
12. Anderson LJ, Holden S, Davis B, et al. Cardiovascular T2-star (T2*) magnetic resonance for the early diagnosis of myocardial iron overload. Eur Heart J 2001 22 : 2171 2179.
13. Wood JC, Enriquez C, Ghugre N, et al. MRI R2 and R2* mapping accurately estimates hepatic iron concentration in transfusion-dependent thalassemia and sickle cell disease patients. Blood 2005 106 : 1460 1465.
14. Cunningham MJ, Macklin EA, Neufeld EJ, Cohen AR. Complications of beta-thalassemia major in North America. Blood 2004 104 : 34 9.
15. Hosmer DW, Lemeshow S. Applied Logistic Regression Analysis. New York : Wiley-Interscience, John Wiley & Sons Inc, 2000.
16. Pennell DJ, Berdoukas V, Karagiorga M, et al. Randomized controlled trial of deferiprone or deferoxamine in beta-thalassemia major patients with asymptomatic myocardial siderosis. Blood 2006 107 : 3738 3744.
17. Kell DB. Iron behaving badly: inappropriate iron chelation as a major contributor to the aetiology of vascular and other progressive inflammatory and degenerative diseases. BMC Med Genomics 2009 2 : 2.
18. Maggio A, Vitrano A, Capra M, et al. 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 Cells Mol Dis 2009 42 : 247 251.
19. Tanner MA, Galanello R, Dessi C, et al. A randomized, placebo-controlled, double-blind trial of the effect of combined therapy with deferoxamine and deferiprone on myocardial iron in thalassemia major using cardiovascular magnetic resonance. Circulation 2007 115 : 1876 1884.
20. ® reduces cardiac iron burden in chronically transfused β-thalassemia patients: An MRI T2* study. Blood (ASH Annual Meeting Abstracts) 2007 110 : 2781.
21. Aessopos A, Fragodimitri C, Karabatsos F, et al. Cardiac magnetic resonance imaging R2* assessments and analysis of historical parameters in patients with transfusion-dependent thalassemia. Haematologica 2007 92 : 131 132. (Pubitemid 46232670)
22. Tanner MA, Galanello R, Dessi C, et al. Myocardial iron loading in patients with thalassemia major on deferoxamine chelation. J Cardiovasc Magn Reson 2006 8 : 543 547.
23. Davis BA, Porter JB. Long-term outcome of continuous 24-hour deferoxamine infusion via indwelling intravenous catheters in high-risk beta-thalassemia. Blood 2000 95 : 1229 1236.
24. Anderson LJ, Westwood MA, Holden S, et al. Myocardial iron clearance during reversal of siderotic cardiomyopathy with intravenous desferrioxamine: a prospective study using T2* cardiovascular magnetic resonance. Br J Haematol 2004 127 : 348 355.
25. Miskin H, Yaniv I, Berant M, Hershko C, Tamary H. Reversal of cardiac complications in thalassemia major by long-term intermittent daily intensive iron chelation. Eur J Haematol 2003 70 : 398 403.
26. Tanner MA, Galanello R, Dessi C, et al. Combined chelation therapy in thalassemia major for the treatment of severe myocardial siderosis with left ventricular dysfunction. J Cardiovasc Magn Reson 2008 10 : 12.
27. Wu KH, Chang JS, Tsai CH, Peng CT. Combined therapy with deferiprone and desferrioxamine successfully regresses severe heart failure in patients with beta-thalassemia major. Ann Hematol 2004 83 : 471 473.
28. Tsironi M, Deftereos S, Andriopoulos P, Farmakis D, Meletis J, Aessopos A. Reversal of heart failure in thalassemia major by combined chelation therapy: a case report. Eur J Haematol 2005 74 : 84 5.
29. Tavecchia L, Masera N, Russo P, et al. Successful recovery of acute hemosiderotic heart failure in beta-thalassemia major treated with a combined regimen of desferrioxamine and deferiprone. Haematologica 2006 91 : ECR19.
30. Berdoukas V, Chouliaras G, Moraitis P, Zannikos K, Berdoussi E, Ladis V. The efficacy of iron chelator regimes in reducing cardiac and hepatic iron in patients with thalassaemia major: a clinical observational study. J Cardiovasc Magn Reson 2009 11 : 20.