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Meta-Analysis
. 2013 Aug 21;2013(8):CD004450.
doi: 10.1002/14651858.CD004450.pub3.

Desferrioxamine mesylate for managing transfusional iron overload in people with transfusion-dependent thalassaemia

Sheila A Fisher  1 , Susan J Brunskill, Carolyn Doree, Sarah Gooding, Onima Chowdhury, David J Roberts
Affiliations
Meta-Analysis

Desferrioxamine mesylate for managing transfusional iron overload in people with transfusion-dependent thalassaemia

Sheila A Fisher et al. Cochrane Database Syst Rev. .

Abstract

Background: Thalassaemia major is a genetic disease characterised by a reduced ability to produce haemoglobin. Management of the resulting anaemia is through red blood cell transfusions.Repeated transfusions result in an excessive accumulation of iron in the body (iron overload), removal of which is achieved through iron chelation therapy. Desferrioxamine mesylate (desferrioxamine) is one of the most widely used iron chelators. Substantial data have shown the beneficial effects of desferrioxamine, although adherence to desferrioxamine therapy is a challenge. Alternative oral iron chelators, deferiprone and deferasirox, are now commonly used. Important questions exist about whether desferrioxamine, as monotherapy or in combination with an oral iron chelator, is the best treatment for iron chelation therapy.

Objectives: To determine the effectiveness (dose and method of administration) of desferrioxamine in people with transfusion-dependent thalassaemia.To summarise data from trials on the clinical efficacy and safety of desferrioxamine for thalassaemia and to compare these with deferiprone and deferasirox.

Search methods: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's Haemoglobinopathies Trials Register. We also searched MEDLINE, EMBASE, CENTRAL (The Cochrane Library), LILACS and other international medical databases, plus ongoing trials registers and the Transfusion Evidence Library (www.transfusionevidencelibrary.com). All searches were updated to 5 March 2013.

Selection criteria: Randomised controlled trials comparing desferrioxamine with placebo, with another iron chelator, or comparing two schedules or doses of desferrioxamine, in people with transfusion-dependent thalassaemia.

Data collection and analysis: Six authors working independently were involved in trial quality assessment and data extraction. For one trial, investigators supplied additional data upon request.

Main results: A total of 22 trials involving 2187 participants (range 11 to 586 people) were included. These trials included eight comparisons between desferrioxamine alone and deferiprone alone; five comparisons between desferrioxamine combined with deferiprone and deferiprone alone; eight comparisons between desferrioxamine alone and desferrioxamine combined with deferiprone; two comparisons of desferrioxamine with deferasirox; and two comparisons of different routes of desferrioxamine administration (bolus versus continuous infusion). Overall, few trials measured the same or long-term outcomes. Seven trials reported cardiac function or liver fibrosis as measures of end organ damage; none of these included a comparison with deferasirox.Five trials reported a total of seven deaths; three in patients who received desferrioxamine alone, two in patients who received desferrioxamine and deferiprone. A further death occurred in a patient who received deferiprone in another who received deferasirox alone. One trial reported five further deaths in patients who withdrew from randomised treatment (deferiprone with or without desferrioxamine) and switched to desferrioxamine alone.One trial planned five years of follow up but was stopped early due to the beneficial effects of a reduction in serum ferritin levels in those receiving combined desferrioxamine and deferiprone treatment compared with deferiprone alone. The results of this and three other trials suggest an advantage of combined therapy with desferrioxamine and deferiprone over monotherapy to reduce iron stores as measured by serum ferritin. There is, however, no evidence for the improved efficacy of combined desferrioxamine and deferiprone therapy against monotherapy from direct or indirect measures of liver iron.Earlier trials measuring the cardiac iron load indirectly by measurement of the magnetic resonance imaging T2* signal had suggested deferiprone may reduce cardiac iron more quickly than desferrioxamine. However, meta-analysis of two trials showed a significantly lower left ventricular ejection fraction in patients who received desferrioxamine alone compared with those who received combination therapy using desferrioxamine with deferiprone.Adverse events were recorded by 18 trials. These occurred with all treatments, but were significantly less likely with desferrioxamine than deferiprone in one trial, relative risk 0.45 (95% confidence interval 0.24 to 0.84) and significantly less likely with desferrioxamine alone than desferrioxamine combined with deferiprone in two other trials, relative risk 0.33 (95% confidence interval 0.13 to 0.84). In particular, four studies reported permanent treatment withdrawal due to adverse events from deferiprone; only one of these reported permanent withdrawals associated with desferrioxamine. Adverse events also occurred at a higher frequency in patients who received deferasirox than desferrioxamine in one trial. Eight trials reported local adverse reactions at the site of desferrioxamine infusion including pain and swelling. Adverse events associated with deferiprone included joint pain, gastrointestinal disturbance, increases in liver enzymes and neutropenia; adverse events associated with deferasirox comprised increases in liver enzymes and renal impairment. Regular monitoring of white cell counts has been recommended for deferiprone and monitoring of liver and renal function for deferasirox.In summary, desferrioxamine and the oral iron chelators deferiprone and deferasirox produce significant reductions in iron stores in transfusion-dependent, iron-overloaded people. There is no evidence from randomised clinical trials to suggest that any one of these has a greater reduction of clinically significant end organ damage, although in two trials, combination therapy with desferrioxamine and deferiprone showed a greater improvement in left ventricular ejection fraction than desferrioxamine used alone.

Authors' conclusions: Desferrioxamine is the recommended first-line therapy for iron overload in people with thalassaemia major and deferiprone or deferasirox are indicated for treating iron overload when desferrioxamine is contraindicated or inadequate. Oral deferasirox has been licensed for use in children aged over six years who receive frequent blood transfusions and in children aged two to five years who receive infrequent blood transfusions. In the absence of randomised controlled trials with long-term follow up, there is no compelling evidence to change this conclusion.Worsening iron deposition in the myocardium in patients receiving desferrioxamine alone would suggest a change of therapy by intensification of desferrioxamine treatment or the use of desferrioxamine and deferiprone combination therapy.Adverse events are increased in patients treated with deferiprone compared with desferrioxamine and in patients treated with combined deferiprone and desferrioxamine compared with desferrioxamine alone. People treated with all chelators must be kept under close medical supervision and treatment with deferiprone or deferasirox requires regular monitoring of neutrophil counts or renal function respectively. There is an urgent need for adequately-powered, high-quality trials comparing the overall clinical efficacy and long-term outcomes of deferiprone, deferasirox and desferrioxamine.

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Conflict of interest statement

None known.

Figures

1
1
PRISMA study flow diagram.
2
2
Risk of bias summary: review authors' judgements about each risk of bias item for each included study.
3
3
Forest plot of comparison: 1 DFO alone versus deferiprone alone, outcome: 1.14 Adverse events.
4
4
Forest plot of comparison: 3 DFO alone versus DFO and deferiprone in combination, outcome: 3.11 Adverse events.
1.1
1.1. Analysis
Comparison 1 DFO alone versus deferiprone alone, Outcome 1 Left ventricular ejection fraction: mean change from baseline (%).
1.2
1.2. Analysis
Comparison 1 DFO alone versus deferiprone alone, Outcome 2 Right ventricular ejection fraction: mean at endpoint (%).
1.3
1.3. Analysis
Comparison 1 DFO alone versus deferiprone alone, Outcome 3 Liver fibrosis Ishak score: mean at endpoint.
1.4
1.4. Analysis
Comparison 1 DFO alone versus deferiprone alone, Outcome 4 Serum ferritin concentration: mean change from baseline (ng/ml).
1.5
1.5. Analysis
Comparison 1 DFO alone versus deferiprone alone, Outcome 5 Urinary iron excretion: mean at endpoint (mg/24h).
1.6
1.6. Analysis
Comparison 1 DFO alone versus deferiprone alone, Outcome 6 Urinary iron excretion: mean over study (%).
1.7
1.7. Analysis
Comparison 1 DFO alone versus deferiprone alone, Outcome 7 Urinary iron excretion: mean change from baseline (mg/24h).
1.8
1.8. Analysis
Comparison 1 DFO alone versus deferiprone alone, Outcome 8 Liver iron concentration: ratio of geometric means at endpoint (mg/g dry weight).
1.9
1.9. Analysis
Comparison 1 DFO alone versus deferiprone alone, Outcome 9 Liver iron concentration: mean change from baseline (mg/g dry weight).
1.10
1.10. Analysis
Comparison 1 DFO alone versus deferiprone alone, Outcome 10 Myocardial T2*: ratio of geometric means of change from baseline.
1.11
1.11. Analysis
Comparison 1 DFO alone versus deferiprone alone, Outcome 11 Chelation efficiency (%).
1.12
1.12. Analysis
Comparison 1 DFO alone versus deferiprone alone, Outcome 12 Plasma NTBI: mean change from baseline (mM).
1.13
1.13. Analysis
Comparison 1 DFO alone versus deferiprone alone, Outcome 13 Total iron excretion: mean at endpoint (mg/kg/day).
1.14
1.14. Analysis
Comparison 1 DFO alone versus deferiprone alone, Outcome 14 Adverse events.
1.15
1.15. Analysis
Comparison 1 DFO alone versus deferiprone alone, Outcome 15 Participant compliance (%).
2.1
2.1. Analysis
Comparison 2 DFO and deferiprone in combination compared with deferiprone alone, Outcome 1 Left ventricular ejection fraction: mean at endpoint (%).
2.2
2.2. Analysis
Comparison 2 DFO and deferiprone in combination compared with deferiprone alone, Outcome 2 Serum ferritin concentration: mean at endpoint (ng/ml).
2.3
2.3. Analysis
Comparison 2 DFO and deferiprone in combination compared with deferiprone alone, Outcome 3 Serum ferritin concentration: mean change from baseline (ng/ml).
2.4
2.4. Analysis
Comparison 2 DFO and deferiprone in combination compared with deferiprone alone, Outcome 4 Urinary iron excretion: mean over study (%).
2.5
2.5. Analysis
Comparison 2 DFO and deferiprone in combination compared with deferiprone alone, Outcome 5 Urinary iron excretion: mean at endpoint (mg/24h).
2.6
2.6. Analysis
Comparison 2 DFO and deferiprone in combination compared with deferiprone alone, Outcome 6 Liver iron concentration: mean at endpoint (mg/g dry weight).
2.7
2.7. Analysis
Comparison 2 DFO and deferiprone in combination compared with deferiprone alone, Outcome 7 Chelation efficiency (%).
2.8
2.8. Analysis
Comparison 2 DFO and deferiprone in combination compared with deferiprone alone, Outcome 8 Plasma NTBI: mean change from baseline (mM).
2.9
2.9. Analysis
Comparison 2 DFO and deferiprone in combination compared with deferiprone alone, Outcome 9 Total Iron excretion: mean at endpoint (mg/kg/day).
2.10
2.10. Analysis
Comparison 2 DFO and deferiprone in combination compared with deferiprone alone, Outcome 10 Adverse Events.
3.1
3.1. Analysis
Comparison 3 DFO alone versus DFO and deferiprone in combination, Outcome 1 Left ventricular ejection fraction: mean at endpoint (%).
3.2
3.2. Analysis
Comparison 3 DFO alone versus DFO and deferiprone in combination, Outcome 2 Serum ferritin concentration: ratio of geometric means at endpoint (ng/ml).
3.3
3.3. Analysis
Comparison 3 DFO alone versus DFO and deferiprone in combination, Outcome 3 Serum ferritin concentration: ratio of geometric means at endpoint (ng/ml).
3.4
3.4. Analysis
Comparison 3 DFO alone versus DFO and deferiprone in combination, Outcome 4 Serum ferritin concentration: mean change from baseline (ng/ml).
3.5
3.5. Analysis
Comparison 3 DFO alone versus DFO and deferiprone in combination, Outcome 5 Urinary iron excretion: mean at endpoint (mg/24h).
3.6
3.6. Analysis
Comparison 3 DFO alone versus DFO and deferiprone in combination, Outcome 6 Urinary iron excretion: mean over study (%).
3.7
3.7. Analysis
Comparison 3 DFO alone versus DFO and deferiprone in combination, Outcome 7 Liver iron concentration: mean change from baseline.
3.8
3.8. Analysis
Comparison 3 DFO alone versus DFO and deferiprone in combination, Outcome 8 Chelation efficency (%).
3.9
3.9. Analysis
Comparison 3 DFO alone versus DFO and deferiprone in combination, Outcome 9 Plasma NBTI: mean change from baseline (mM).
3.10
3.10. Analysis
Comparison 3 DFO alone versus DFO and deferiprone in combination, Outcome 10 Total iron excretion: mean at endpoint (mg/kg/day).
3.11
3.11. Analysis
Comparison 3 DFO alone versus DFO and deferiprone in combination, Outcome 11 Adverse events.
4.1
4.1. Analysis
Comparison 4 DFO compared with Deferasirox, Outcome 1 Serum ferritin concentration: mean change from baseline (ng/ml).
4.2
4.2. Analysis
Comparison 4 DFO compared with Deferasirox, Outcome 2 Liver iron concentration: mean change from baseline (mg/g dry weight).
4.3
4.3. Analysis
Comparison 4 DFO compared with Deferasirox, Outcome 3 Total iron excretion: ratio of iron excretion to iron intake (mg/kg/day).
5.1
5.1. Analysis
Comparison 5 DFO schedule A (either method of administration or dose A) compared with DFO schedule B (either method of administration or dose B), Outcome 1 Serum ferritin concentration: mean at endpoint (ng/ml).
5.2
5.2. Analysis
Comparison 5 DFO schedule A (either method of administration or dose A) compared with DFO schedule B (either method of administration or dose B), Outcome 2 Urinary iron excretion (mg/24h).
5.3
5.3. Analysis
Comparison 5 DFO schedule A (either method of administration or dose A) compared with DFO schedule B (either method of administration or dose B), Outcome 3 Liver iron concentration: mean at endpoint (mg/g dry weight).
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    1. Aldouri MA, Wonke B, Hoffbrand AV, Flynn DM, Ward SE, Agnew JE, et al. High incidence of cardiomyopathy in beta‐thalassaemia patients receiving regular transfusion and iron chelation: reversal by intensified chelation. Acta Haematologica 1990;84(3):113‐7. - PubMed
al Refaie 1992 {published data only}
    1. Al‐Refaie FN, Wickens DG, Wonke B, Kontoghiorghes GJ, Hoffbrand AV. Serum non‐transferrin‐bound iron in beta‐thalassaemia major patients treated with desferrioxamine and L1. British Journal of Haematology 1992;82:431‐6. - PubMed
Anderson 2002 {published data only}
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Andres 1980 {published data only}
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Athanassiou‐Metaxa 2004 {published data only}
    1. Athanassiou‐Metaxa M, Kousi A, Hatzipantelis E, Tsatra I, Ikonomou M, Perifanis V, et al. Combined chelation therapy with deferiprone and desferrioxamine in iron overloaded ß‐thalassemia patients. Haematologica 2004; Vol. 89, issue 3:ELT07. - PubMed
Barry 1974 {published data only}
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Bartfay 1999 {published data only}
    1. Bartfay WJ, Lehotay DC, Sher GD, Bartfay E, Tyler B, Luo X, et al. Erythropoiesis: comparison of cytotoxic aldehyde generation in beta‐thalassaemia patients chelated with deferoxamine or deferiprone (L1) versus no chelation. Haematology 1999;4(1):67‐76. - PubMed
Borgna‐Pignatti 1989 {published data only}
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Brittenham 2003b {published data only}
    1. Brittenham GM, Griffith PM, Nienhuis AW, McLaren CE, Young NS, Tucker EE. Efficacy of deferoxamine in preventing complications of iron overload in patients with thalassaemia major. New England Journal of Medicine 1994;331(9):567‐73. - PubMed
B‐Weintrob 1990 {published data only}
    1. Bronspiegel‐Weintrob N, Olivieri NF, Tyler B, Andrews DF, Freedman M, Holland J. Effect of age at the start of iron chelation therapy on gonadal function in beta‐thalassaemia major. New England Journal of Medicine 1990;323(11):713‐9. - PubMed
Calleja 1998 {published data only}
    1. Calleja EM, Shen JY, Lesser M, Grady RW, New MI, Giardina PJ. Survival and morbidity in transfusion‐dependent thalassaemic patients on subcutaneous desferrioxamine chelation. Nearly two decades of experience. Annals New York Academy of Science 1998;850:469‐70. - PubMed
Cassinerio 2012 {published data only}
    1. Cassinerio E, Roghi A, Pedrotti P, Brevi F, Zanaboni L, Graziadei G, et al. Cardiac iron removal and functional cardiac improvement by different iron chelation regimens in thalassemia major patients. Annals of Hematology 2012;91(9):1443‐9. - PubMed
Christoforidis 2007 {published data only}
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Cianciulli 1993 {published data only}
    1. Cianciulli P, Forte L, Sorrentino F, Palombi M, Papa G, Marciani MG. Intensive long‐term intravenous iron‐chelation therapy with deferoxamine in thalassaemic patients. Bone Marrow Transplantation 1993;12 Suppl 1:5‐8. - PubMed
Cianciulli 1994 {published data only}
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Davies 1983 {published data only}
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De Sanctis 1994 {published data only}
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DeVirgiliis 1988 {published data only}
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Drakonaki 2010 {published data only}
    1. Drakonaki EE, Maris TG, Maragaki S, Klironomos V, Papadakis A, Karantanas AH. Deferoxamine versus combined therapy for chelating liver, spleen and bone marrow iron in beta‐thalassemic patients: a quantitative magnetic resonance imaging study. Hemoglobin 2010;34(1):95‐106. - PubMed
Elalfy 2006 {published data only}
    1. Elalfy MS, Abdin I. Liver status in a cohort of polytransfused b‐thalasseamie major (BTM) on long term desferrioxamine (DFO) or Deferiprone (L1) [abstract]. Blood 2006;108:Abstract no: 3732.
Eleftheriou 2006 {published data only}
    1. Eleftherioiu P, Tanner M, Pennell D, Porter J. Response of myocardial T2* to oral deferasirox monotherapy for 1 year in 29 patients with transfusion‐dependent anaemias: a subgroup analysis [abstract]. Haematologica 2006;91 Suppl 1:Abstract no: 366.
    1. Porter KJB, Tanner MA, Pennell DJ, Eleftheriou P. Improved myocardial mT2* in transfusion dependent anemias receiving ICL670 (Deferasirox) [abstract]. Blood 2005;106:Abstract no: 3600.
Fragatou 2007 {published data only}
    1. Fragatou S, Politis C, Vandiadi K, Tsiapras D, Douskou M. Cardiac function in thalassaemics on combined deferoxamine and deferiprone therapy [abstract]. Haematologica 2007;92:299, Abstract no: 0801.
Galanello 1999 {published data only}
    1. Galanello R, Kattamis C, Athanassiou M, Quarta G, Ballati G, Zoumbos N, et al. A depot formulation of desferrioxamine (ICL749B): update on the dose‐finding program [abstract]. Blood 1999;94:32b.
Gaziev 1995 {published data only}
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Gharagozloo 2009 {published data only}
    1. Gharagozloo M, Moayedi B, Zakerinia M, Hamidi M, Karimi M, Maracy M, et al. Combined therapy of silymarin and desferrioxamine in patients with beta‐thalassemia major: a randomized double‐blind clinical trial. Fundamental and Clinical Pharmacology 2009;23(3):359‐65. - PubMed
Goulas 2012 {published data only}
    1. Goulas V, Kourakli‐Symeonidis A, Camoutisis C. Comparative effects of three iron chelation therapies on the quality of life in Greek patients with homozygous transfusion‐dependent beta‐thalassemia. http://www.hindawi.com/isrn/hematology/2012/139862/cta/ (accessed 01 August 2013). - PMC - PubMed
Grady 2001 {published data only}
    1. Grady RW, Berdoukas V, Rachmilewitz EA, Galanello R, Borgna‐Pignatti C, Ladis V. When deferiprone and desferrioxamine are combined iron excretion is enhanced [abstract]. Blood 2001;98(11 Pt 1):494a.
Graziano 1978 {published data only}
    1. Graziano JH, Markenson A, Miller DR, Chang H, Bestak M, Meyers P, et al. Chelation therapy in beta‐thalassaemia major. I. Intravenous and subcutaneous deferoxamine. Journal of Pediatrics 1978;92(4):648‐52. - PubMed
Hussain 1976 {published data only}
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Kattamis 1981 {published data only}
    1. Kattamis C, Fitsialos J, Sinopoulou C. Oral desferrioxamine in young patients with thalassaemia. Lancet 1981;1(8210):51. - PubMed
Kattamis 2003 {published data only}
    1. Kattamis A, Kassou C, Berdousi H, Ladis V, Papassotiriou I, Kattamis C. Combined therapy with desferrioxamine and deferiprone in thalassemic patients: effect on urinary iron excretion. Haematologica 2003;88(12):1423‐5. - PubMed
Keshtkaran 2013 {published data only}
    1. Keshtharan A, Javanbakht M, Salavati S, Mashayekhi A, Karimi M, Nuri B. Cost‐utility analysis of oral deferasirox versus infusional deferoxamine in transfusion‐dependent beta‐thalassemia patients. Transfusion 2013;53(8):1722‐9. [DOI: 10.1111/trf.12024] - DOI - PubMed
Kontoghiorghes 1987 {published data only}
    1. Kontoghiorghes GJ, Aldouri MA, Hoffbrand AV, Barr J, Wonke B, Kourouclaris T, et al. Effective chelation of iron in beta thalassaemia with the oral chelator 1,2‐dimethyl‐3‐hydroxypyrid‐4‐one.. British Medical Journal (Clinical Research Edition) 1987;295(6612):1509‐12. - PMC - PubMed
Lai 2010 {published data only}
    1. Lai ME, Grady RW, Vacquer S, Pepe A, Carta MP, Bina P, et al. Increased survival and reversion of iron‐induced cardiac disease in patients with thalassemia major receiving intensive combined chelation therapy as compared to deferoxamine alone. Blood Cells, Molecules and Diseases 2010;45(2):136‐9. - PubMed
Li 2000 {published data only}
    1. Li CK, Lai DH, Shing MMK, Chik KW, Lee V, Yuen PMP. Early iron reduction programme for thalassaemia patients after bone marrow transplantation. Bone Marrow Transplantation 2000;25(6):653‐6. - PubMed
Loebstein 1997 {published data only}
    1. Loebstein R, Dalal I, Nisbet‐Brown E, Berkovitch M, Meydan N, Andrews D, et al. Immune function in patients with beta‐thalassamia receiving the orally active iron‐chelating agent deferiprone. British Journal of Haematology 1997;98(3):597‐600. - PubMed
Nienhuis 1976 {published data only}
    1. Nienhuis AW, Delea C, Aamodt R, Bartter F, Anderson WF. Evaluation of desferrioxamine and ascorbic acid for the treatment of chronic iron overload. Birth Defects: Original Article Series 1976;12(8):177‐85. - PubMed
Olivieri 1992 {published data only}
    1. Olivieri NF, Berriman AM, Tyler BJ, Davis SA, Francombe WH, Liu PP. Reduction in tissue iron stores with a new regimen of continuous ambulatory intravenous deferoxamine. American Journal of Haematology 1992;41(1):61‐3. - PubMed
Peng 2006 {published data only}
    1. Peng CT, Wu KH, Tsai CC, Ysai CH. Deferirpone in patients with beta‐thalassamiea major for 4 years in the Chinese population in Taiwan [abstract]. European Journal of Clinical Investigation 2004;34 Suppl 1:20‐61, Abstract no:138.
    1. Peng CT, Wu KH, Tsai CH, Yang CP, Wang LW, Jang RC, et al. Study of deferiprone or deferoxamine versus combination therapy in iron‐loaded thalassaemia patients in Taiwan [abstract]. Blood 2006;18:Abstract no: 3736.
Pennell 2012 {published data only}
    1. Pennell D, Porter JB, Cappellini MD, Chan L, El‐Beshlawi A, Aydinok Y, et al. Continued improvement and normalization of myocardial T2* in patients with beta‐thalassemia major treated with deferasirox (Exjade) for up to 3 years. Blood. 2010; Vol. 116 (21).
    1. Pennell DJ, Porter JB, Cappellini MD, Chan LL, El‐Beshlawi A, Aydinok Y. Deferasirox for up to 3 years leads to continued improvement of myocardial T2* in patients with beta‐thalassemia major. Haematologica 2012;97(6):842‐8. - PMC - PubMed
Pepe 2006 {published data only}
    1. Pepe A, Lombardi M, Positano V, Cracolici E, Capra M, Malizia R, et al. Evaluation of the efficacy of oral deferiprone in beta‐thalassaemia major by multislice multiecho T2*. European Journal of Haematology 2006;76(3):183‐92. - PubMed
Piga 2007 {published data only}
    1. Piga A, Vichinsky E, Forni GL, Killinc Y, Maseruka H, Kattamis A. Long‐term efficacy and safety with Deferasirox (Exjade ICL670) a once‐daily oral iron chelator in pediatric patients [abstract]. Blood 2007;110:Abstract no: 2774.
Pippard 1978b {published data only}
    1. Pippard MJ, Callender ST, Weatherall DJ. Intensive iron‐chelation therapy with desferrioxamine in iron‐loading anaemias. Clinical Science and Molecular Medicine 1978;54(1):99‐106. - PubMed
Propper 1977 {published data only}
    1. Propper RD, Cooper B, Rufo R, Nienhuis AW, Anderson WF, Bunn HF. Continuous subcutaneous administration of deferoxamine in patients with iron overload. New England Journal of Medicine 1977;297(8):418‐23. - PubMed
Ricchi 2010 {published data only}
    1. Ricchi P, Ammirabile M, Spasiano A, Costantini S, Cinque P, Matola T, et al. Combined chelation therapy in thalassemia major with deferiprone and desferrioxamine: a retrospective study. European Journal of Haematology 2010;85(1):36‐42. - PubMed
Russo 1990 {published data only}
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Tanner 2008 {published data only}
    1. Tanner MA, Galanello R, Dessi C, Smirth GC, Westwood MA, Agus A, et al. Combined chelation therapy in thalassaemia major for the treatment of severe myocardial siderosis with left ventricular dysfunction. Journal of Cardiovascular Magnetic Resonance 2008;10(1):12‐20. - PMC - PubMed
Torcharus 1993 {published data only}
    1. Torcharus K, Withayathawornwong W, Sriphaisal T, Krutvacho T, Arnutti P, Suwanasophorn C. High transfusion in children with beta‐thalassaemia/Hb E: clinical and laboratory assessment of 18 cases. Southeast Asian Journal of Tropical Medicine and Public Health 1993;24 Suppl 1:96‐9. - PubMed
Tsakok 2004 {published data only}
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Vannasaeng 1991 {published data only}
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Vlachaki 2007 {published data only}
    1. Vlachaki E, Ioannidou‐Papagiannaki E, Haralambidou‐Vranitsa SH, Perifanis V, Tsigga A, Klonizakis I, et al. Progenitor haemopoietic cells in the peripheral blood of thalassemic patients with desferrioxamine or deferiprone chelation therapy [abstract]. Hematology 2005;90 Suppl 2:Abstract no: 1065.
    1. Vlachaki E, Ioannidou‐Papagiannaki E, Tziomalos K, Haralambidou‐Vranitsa S, Perifanis V, Klonizakis I, et al. Peripheral blood haematopoietic progenitor cells in patients with beta thalassaemia major receiving desferrioxamine or deferiprone as chelation therapy. European Journal of Haematology 2007;78(1):48‐51. - PubMed
Walter 2008 {published data only}
    1. Walter PB, Macklin EA, Porter J, Evans P, Kwiatkowski JL, Neufeld EJ, et al. Inflamation and oxidant stress in beta‐thalassaemia patients treated with iron chelators deferasirox (ICL670) or deferoxamine: an ancillary study of Novartis CICL670A0107. Haematologica 2008;93(6):817‐25. - PubMed
Wang 2006 {published data only}
    1. Wang CH, Wu KH, T FJ, Peng CT, T CH. Comparison of oral and subcutaneous iron chelation therapies in the prevention of major endocrinopathies in beta‐thalassaemia major patients. Hemoglobin 2006;30(2):257‐62. - PubMed
Wonke 1998 {published data only}
    1. Wonke B, Wright C, Hoffbrand AV. Combined therapy with deferiprone and desferrioxamine. British Journal of Haematology 1998;103(2):361‐4. - PubMed
Zareifar 2009 {published data only}
    1. Zareifar S, Jabbari A, Cohan N, Haghpanah S. Efficacy of combined desferrioxamine and deferiprone versus single desferrioxamine therapy in patients with major thalassemia. Archives of Iranian Medicine 2009;12(5):488‐91. - PubMed

References to studies awaiting assessment

Alpendurada 2012 {published data only}
    1. Alpendurada F, Smith GC, Carpenter JP, Nair SV, Tanner MA, Banya W, et al. Effects of combined deferiprone with deferoxamine on right ventricular function in thalassemia major. Journal of Cardiovascular Magnetic Resonance 2012;14(1):8. - PMC - PubMed
Aydinok 2012 {published data only}
    1. Aydinok Y, Evans P, Manz CY, Porter JB. Probing the origin of chelatable iron during deferiprone and combination therapies: insights from plasma NTBI and LPI determinations [abstract]. Blood 2010;116(21):Abstract no: 5158.
    1. Aydinok Y, Evans P, Manz CY, Porter JB. Timed non‐transferrin bound iron determinations probe the origin of chelatable iron pools during deferiprone regimens and predict chelation response. Haematologica 2012;97(6):835‐41. - PMC - PubMed
Badawi 2010 {published data only}
    1. Badawy S, Hassan TH, Hesham MAA, Badr MA. Evaluation of iron chelation therapy in beta‐thalassemic patients in Zagazig University Hospital. Pediatric Blood and Cancer; Proceedings of the 23rd Annual Meeting of the American Society of Pediatric Hematology/Oncology, ASPHO, Montreal, Canada. 2010:799‐800, Poster no: 124.
    1. Hassan T, Badr M, Hesham M, Badawy S. Evaluation of iron chelation therapy in beta‐thalassemia major patients in East Delta of Egypt. Haematologica 2010;95 Suppl 2:701, Abstract no: 1810.
Canatan 1999 {published data only}
    1. Canatan D, Temimhan N, Dincer N, Ozsancak A, Oguz N, Temimhan M. Continuous desferrioxamine infusion by an infusor in thalassaemia major. Acta Paediatrica 1999;88(5):550‐2. - PubMed
Evans 2011 {published data only}
    1. Evans P, Aydinok Y, Manz C, Porter J. Origin of chelatable iron during deferiprone and combination therapies: Insights from plasma NTBI and LPI. American Journal of Hematology 2011;86:9: E84.
Jain 2011 {published data only}
    1. Jain R, Perkins J, Johnson S, Harimoorthy V, Desai P, Chudgar U, et al. A prospective study for determination of the mean red cell transfusion requirement compared on the basis of iron overload and type of chelation therapy in multiply transfused thalassaemia major patients. Transfusion Medicine. 29th Annual Scientific Meeting of the British Blood Tranfusion Society, Glasgow, UK 2011;21:46‐7.
Kompany 2009 {published data only}
    1. Kompany F, Mohammadi S, Sigari N, Hadizadeh N, Rezaie N, Gharibi FSM. Comparative efficacy of deferrioxamine and combination of deferiprone and deferrioxamine on echocardiographic indices in beta thalassemic patients. Scientific Journal of Kurdistan University of Medical Sciences 2009;14(2):21‐30.
Maggio 2012 {published data only}
    1. Maggio A, Capra M, Cuccia L, Gagliardotto F, Rigano P, Calvaruso G, et al. Long‐term use of deferiprone enhances significantly the left ventricular ejection function in thalassemia major [abstract]. ASH Annual Meeting Abstracts; 53rd American Society of Hematology (ASH) Annual Meeting, 10‐13 December 2011, San Diego, USA. 2011; Vol. 118:21; Abstract no: 5302.
    1. Maggio A, Vitrano A, Lucania G, Capra M, Cuccia L, Gagliardotto F, et al. Long‐term use of deferiprone significantly enhances left‐ventricular ejection function in thalssemia major patients. American Journal of Hematology 2012;87(7):732‐3. - PubMed
Mirbehbahani 2012 {published data only}
    1. Mirbehbahani N, Jahazi A, Rahim AHH. The effect of combined therapy with deferoxamine and deferiprone on serum ferritin level of beta‐thalassemic patients. Hematology 2012;17(3):183‐6. - PubMed
N0277104959 {published data only}
    1. N0277104959. A randomised controlled prospective trial using Ferriprox versus placebo and conventional intravenous and subcutaneous iron chelation. http://www.nihr.ac.uk/Profile/Pages/NRRResults.aspx?publication_id=N0277... (accessed 1 September 2009).
NCT00004982 {published data only}
    1. NCT00004982. Combination Iron Chelation Therapy. http://www.clinicaltrials.gov/ct2/show/NCT00004982?term=NCT00004982&... (accessed 5 September 2011).
NCT00115349 {published data only}
    1. NCT00115349. Combination therapy compared with single‐drug therapy in patients with cardiac diseases. http://www.clinicaltrials.gov/ct2/show/NCT00115349?term=NCT00115349&... (accessed 5 September 2011).
Pantalone 2011 {published data only}
    1. Pantalone GR, Maggio A, Vitrano A, Capra M, Cuccia L, Gagliardotto F, et al. Sequential alternating deferiprone and deferoxamine treatment compared to deferiprone monotherapy: Main findings and clinical follow‐up of a large multicenter randomized clinical trial in beta‐thalassemia major patients. Hemoglobin 2011;35(3):206‐16. - PubMed
Pennell 2010 {published data only}
    1. Pennell D, Porter J, Piga A, El‐Alfy M, El‐Beshlawi A, Kilinc Y, et al. Prevalence of cardiac iron overload in patients with transfusion‐dependent anemias: data from the ranodomized, active‐controlled deferasirox CORDELIA trial. Haematologica. 17th Congress of the European Hematology Assocation, Amsterdam, The Netherlands, 14‐17 June 2012. 2012; Vol. 97 (S1):Abstract no: 0927.
    1. Pennell DJ, Porter JB, Piga A, Lai Y, El‐Beshlawi A, Beloul K, et al. A multicenter, randomized, open‐label trial evaluating deferasirox compared with deferoxamine for the removal of cardiac iron in patients with beta‐thalassemia major and iron overload (CORDELIA). Blood. 2012; Vol. 121 (21).
Pepe 2013 {published data only}
    1. Pepe A, Meloni A, Pepe P, Capra M, D'Ascola DG, Santodirocco M, et al. Prospective comparison on cardiac and hepatic iron and cardiac function by MR in thalassemia major patients treated with combination deferiprone‐desferrioxamine versus deferiprone and desferrioxamine in monotherapy [abstract]. Blood 2011;118(21):Abstract.
    1. Pepe A, Meloni A, Rossi G, Cuccia L, D'Ascola GD, Santodirocco M, et al. Cardiac and hepatic iron and ejection fraction in thalassemia major: multicentre prospective comparison of combined deferiprone and deferoxamine therapy against deferiprone or deferoxamine monotherapy. http://www.jcmr‐online.com/content/pdf/1532‐429X‐15‐1.pdf (accessed 01 Augsut 2013). [DOI: 10.1186/1532-429X-15-1] - DOI - PMC - PubMed
    1. Pepe A, Meloni A, Rossi G, Ruffo GB, D'Ascola DG, Santodirocco M, et al. Cardiac iron and function by CMR in thalassemia major patients treated with combined deferiprone and desferrioxamine regimen versus montherapies: a multi‐center, observational and prospective study. European Heart Journal 2012;33:805.
    1. Pepe A, Rossi G, Meloni A, Dell'Amico MC, Capra M, Caruso V, et al. Prospective comparison on cardiac iron and liver iron by MR in thalassemia major patients treated with combination deferiprone‐desferrioxamine versus deferiprone and desferrioxamine in monotherapy [abstract]. Blood 2010;116(21):Abstract no: 5164.
    1. Pepe A, Rossi G, Meloni A, Dell'Amico MC, Capra M, Caruso V, et al. Prospective comparison on cardiac iron and liver iron by MR in thalassemia major patients treated with combination deferiprone‐desferrioxamine versus deferiprone and desferrioxamine in monotherapy [abstract]. Haematologica, Proceedings of the 15th Congress of the European Hematology Association, Barcelona, Spain 2010;95 Suppl 2:696, Abstract no: 1797.
Unal 2009 {published data only}
    1. Unal S, Hazirolan T, Beton B, Karabulut E, Gumruk F. The cardiac effects of desferoxamine deferiprone combination therapy and desferoxamine monotherapy in thalassemic patients [abstract]. Haematologica 2009;94 Suppl 2:514‐5, Abstract no: 1295.

References to ongoing studies

IRCT201110087677N1 {published data only}
    1. IRCT20110087677N1. The comparative study of incidence of lens opacity between Osfereal and Deferoxamine in major thalassemia. http://apps.who.int/trialsearch/trial.aspx?trialid=IRCT201110087677N1 (accessed 18 June 2012).
IRCT201206289827N2 {published data only}
    1. IRCT201206289827N2. Comparison of two methods of administration of deferoxamine (intravenous and subcutaneous) in terms of impact on reducing iron overload in thalassemia patients who have suffered heart failure. http://apps.who.int/trialsearch/trial.aspx?trialid=IRCT201206289827N2 (accessed 5 March 2013).
NCT01511848 {published data only}
    1. NCT01511848. Study of efficacy, safety of combined deferasirox and deferiprone versus combined deferiprone and desferal in conditions of iron overload. http://www.clinicaltrials.gov/ct2/show/NCT01511848?term=NCT01511848&... (accessed 18 June 2012).

Additional references

Agarwal 1992
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Argyropoulou 2003
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Aydinok 1999
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Bacon 1983
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Berdoukas 2000
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BNF 2012
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References to other published versions of this review

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