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

Roberts, David J.; Rees, David C.; Howard, Jo; Hyde, Chris; Brunskill, Susan J

doi:10.1002/14651858.cd004450.pub2

Cited by 20 publications

(11 citation statements)

References 77 publications

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“…When administered in adequate doses it reduces iron overload, improves cardiac function and has been shown to reduce iron-related morbidity and mortality. 4 Unfortunately, compliance still remains a serious limiting factor in treatment success because of requirement of subcutaneous infusions and high cost. Vitamin C has been shown to increase the availability of cheatable iron and is given at the time of desferrioxamine infusion in the dose of 2-3 mg/kg/day.…”

Section: Desferrioxamine (Dfo)mentioning

confidence: 99%

Current Trends in the Management of Beta Thalassemia

2008

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Section: Desferrioxamine (Dfo)mentioning

confidence: 99%

Current Trends in the Management of Beta Thalassemia

2008

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“…The ability of desferrioxamine to significantly reduce body iron burden has been repeatedly demonstrated. 29 However, in all the series reported, 65% of the patients treated with desferrioxamine alone were found to have a T2* in the midventricular septum less than 20 ms and, therefore, less than an optimal target. 30 These findings confirm a possible important difference in drug action: deferiprone seems to be a better agent for removing/preventing myocardial siderosis, but less effective in removing hepatocellular…”

mentioning

confidence: 93%

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

Pepe¹,

Meloni²,

Capra³

et al. 2010

Haematologica

127

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BackgroundOral deferiprone was suggested to be more effective than subcutaneous desferrioxamine for removing heart iron. Oral once-daily chelator deferasirox has recently been made commercially available but its long-term efficacy on cardiac iron and function has not yet been established. Our study aimed to compare the effectiveness of deferasirox, deferiprone and desferrioxamine on myocardial and liver iron concentrations and bi-ventricular function in thalassemia major patients by means of quantitative magnetic resonance imaging. Design and MethodsFrom the first 550 thalassemia subjects enrolled in the Myocardial Iron Overload in Thalassemia network, we retrospectively selected thalassemia major patients who had been receiving one chelator alone for longer than one year. We identified three groups of patients: 24 treated with deferasirox, 42 treated with deferiprone and 89 treated with desferrioxamine. Myocardial iron concentrations were measured by T2* multislice multiecho technique. Biventricular function parameters were quantitatively evaluated by cine images. Liver iron concentrations were measured by T2* multiecho technique. ResultsThe global heart T2* value was significantly higher in the deferiprone (34±11ms) than in the deferasirox (21±12 ms) and the desferrioxamine groups (27±11 ms) (P=0.0001). We found higher left ventricular ejection fractions in the deferiprone and the desferrioxamine versus the deferasirox group (P=0.010). Liver iron concentration, measured as T2* signal, was significantly lower in the desferrioxamine versus the deferiprone and the deferasirox group (P=0.004). ConclusionsThe cohort of patients treated with oral deferiprone showed less myocardial iron burden and better global systolic ventricular function compared to the patients treated with oral deferasirox or subcutaneous desferrioxamine.Key words: thalassemia, iron chelation therapy, cardiac magnetic resonance imaging.Citation: Pepe A, Meloni A, Capra M, Cianciulli P, Prossomariti L, Malaventura C, Putti MC, Lippi A, Romeo MA, Bisconte MG, Filosa A, Caruso V, Quarta A, Pitrolo L, Missere M, Midiri M, Rossi G, Positano V, Lombardi M, and Maggio A. Deferasirox, deferiprone

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“…26 Deferasirox is a very effective chelator, but it is not superior to the other chelating drugs. 27 In a recently published study, long term follow-up of both adults and children showed good safety and efficacy of deferasirox in removing liver iron and reversed or stabilised liver fibrosis in 83% of the patients. 28 29 Furthermore, a longitudinal study in 39 patients showed that continuous treatment with deferasirox for two years, with a target dose of 40 mg/kg per day, continued to remove iron from the heart in patients with β thalassemia major and mild, moderate, or severe cardiac siderosis.…”

Section: How Should Iron Overload Be Prevented and Treated?mentioning

confidence: 96%