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The efficacy of iron chelator regimes in reducing cardiac and hepatic iron in patients with thalassaemia major: a clinical observational study.

Berdoukas V, Chouliaras G, Moraitis P, Zannikos K, Berdoussi E, Ladis V - J Cardiovasc Magn Reson (2009)

Bottom Line: For the heart, deferiprone and the combination of deferiprone and deferoxamine significantly reduced cardiac iron at all levels of iron loading.Combination therapy is best in reducing both cardiac and hepatic iron, while monotherapy with deferiprone or deferasirox are effective in the heart and liver respectively.The outcomes of this study may be useful to physicians as to the chelation they should prescribe according to the levels of iron load found in the heart and liver by MR.

View Article: PubMed Central - HTML - PubMed

Affiliation: Thalassaemia Unit, First Department of Paediatrics, University of Athens, Aghia Sophia"Children's Hospital, Athens, Greece. plomari@hol.gr

ABSTRACT

Background: Available iron chelation regimes in thalassaemia may achieve different changes in cardiac and hepatic iron as assessed by MR. The aim of this study was to assess the efficacy of four available iron chelator regimes in 232 thalassaemia major patients by assessing the rate of change in repeated measurements of cardiac and hepatic MR.

Results: For the heart, deferiprone and the combination of deferiprone and deferoxamine significantly reduced cardiac iron at all levels of iron loading. As patients were on deferasirox for a shorter time, a second analysis ("Initial interval analysis") assessing the change between the first two recorded MR results for both cardiac and hepatic iron (minimum interval 12 months) was made. Combination therapy achieved the most rapid fall in cardiac iron load at all levels and deferiprone alone was significantly effective with moderate and mild iron load. In the liver, deferasirox effected significant falls in iron load and combination therapy resulted in the most rapid decline.

Conclusion: With the knowledge of the efficacy of the different available regimes and the specific iron load in the heart and the liver, appropriate tailoring of chelation therapy should allow clearance of iron. Combination therapy is best in reducing both cardiac and hepatic iron, while monotherapy with deferiprone or deferasirox are effective in the heart and liver respectively. The outcomes of this study may be useful to physicians as to the chelation they should prescribe according to the levels of iron load found in the heart and liver by MR.

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Rate of change of T2* according to severity of baseline cardiac iron load and chelation regime prescribed.
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Figure 1: Rate of change of T2* according to severity of baseline cardiac iron load and chelation regime prescribed.

Mentions: The hypothesis that the rate of change of T2* depends on baseline iron level was confirmed by the repeated measurements analysis. The interaction term between the rate of change in T2* and the baseline level of siderosis appears significant in the case of DFO, DFP and Comb, but not in the case of DFX. Based on these results all the comparisons are reported before and after stratification. The estimated annual rates of change in T2*(r-T2*) for each regime according to the severity of the cardiac iron loading (results adjusted for age – gender was not significant), are presented in figure 1. Only DFP or Comb demonstrated significant improvement in cardiac T2* at all levels of cardiac iron loading.


The efficacy of iron chelator regimes in reducing cardiac and hepatic iron in patients with thalassaemia major: a clinical observational study.

Berdoukas V, Chouliaras G, Moraitis P, Zannikos K, Berdoussi E, Ladis V - J Cardiovasc Magn Reson (2009)

Rate of change of T2* according to severity of baseline cardiac iron load and chelation regime prescribed.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2713224&req=5

Figure 1: Rate of change of T2* according to severity of baseline cardiac iron load and chelation regime prescribed.
Mentions: The hypothesis that the rate of change of T2* depends on baseline iron level was confirmed by the repeated measurements analysis. The interaction term between the rate of change in T2* and the baseline level of siderosis appears significant in the case of DFO, DFP and Comb, but not in the case of DFX. Based on these results all the comparisons are reported before and after stratification. The estimated annual rates of change in T2*(r-T2*) for each regime according to the severity of the cardiac iron loading (results adjusted for age – gender was not significant), are presented in figure 1. Only DFP or Comb demonstrated significant improvement in cardiac T2* at all levels of cardiac iron loading.

Bottom Line: For the heart, deferiprone and the combination of deferiprone and deferoxamine significantly reduced cardiac iron at all levels of iron loading.Combination therapy is best in reducing both cardiac and hepatic iron, while monotherapy with deferiprone or deferasirox are effective in the heart and liver respectively.The outcomes of this study may be useful to physicians as to the chelation they should prescribe according to the levels of iron load found in the heart and liver by MR.

View Article: PubMed Central - HTML - PubMed

Affiliation: Thalassaemia Unit, First Department of Paediatrics, University of Athens, Aghia Sophia"Children's Hospital, Athens, Greece. plomari@hol.gr

ABSTRACT

Background: Available iron chelation regimes in thalassaemia may achieve different changes in cardiac and hepatic iron as assessed by MR. The aim of this study was to assess the efficacy of four available iron chelator regimes in 232 thalassaemia major patients by assessing the rate of change in repeated measurements of cardiac and hepatic MR.

Results: For the heart, deferiprone and the combination of deferiprone and deferoxamine significantly reduced cardiac iron at all levels of iron loading. As patients were on deferasirox for a shorter time, a second analysis ("Initial interval analysis") assessing the change between the first two recorded MR results for both cardiac and hepatic iron (minimum interval 12 months) was made. Combination therapy achieved the most rapid fall in cardiac iron load at all levels and deferiprone alone was significantly effective with moderate and mild iron load. In the liver, deferasirox effected significant falls in iron load and combination therapy resulted in the most rapid decline.

Conclusion: With the knowledge of the efficacy of the different available regimes and the specific iron load in the heart and the liver, appropriate tailoring of chelation therapy should allow clearance of iron. Combination therapy is best in reducing both cardiac and hepatic iron, while monotherapy with deferiprone or deferasirox are effective in the heart and liver respectively. The outcomes of this study may be useful to physicians as to the chelation they should prescribe according to the levels of iron load found in the heart and liver by MR.

Show MeSH
Related in: MedlinePlus