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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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Related in: MedlinePlus

Estimated annual rates of change of CIC and R2*. Estimated annual rates of change of CIC (r-CIC, mg/gdw per year) and R2* (sec-1) for each regime at starting T2* levels of 5, 11 and 17 ms (top panel) and quadratic fit curves respectively (bottom panel) as evaluated from the "repeated measurements analysis".
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Figure 2: Estimated annual rates of change of CIC and R2*. Estimated annual rates of change of CIC (r-CIC, mg/gdw per year) and R2* (sec-1) for each regime at starting T2* levels of 5, 11 and 17 ms (top panel) and quadratic fit curves respectively (bottom panel) as evaluated from the "repeated measurements analysis".

Mentions: Due to the non-linear relationship between T2* and CIC, the estimated constant rate of change of T2* within each severity group corresponds to a variable rate of change for CIC within the same severity group. For this reason, we produced estimates of the annual change of CIC for baseline measurements of 5, 11 and 17 ms (Figure 2, top panel). These values represent, more or less, the middle of each severity range. A quadratic fit curve based on many estimations of r-CIC according to a wide range of values of the baseline T2* (2–18 ms) is also presented in Figure 2 for each treatment regime (bottom panel). The observed increasing trend for r-CIC for baseline T2* values > 15 ms is due to the discontinuous form of iron severity grouping which produced overlapping of the estimated r-CIC between the three iron load groups. This would have been less likely to occur if an extremely large number of patients was available, allowing for a very narrow categorization.


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)

Estimated annual rates of change of CIC and R2*. Estimated annual rates of change of CIC (r-CIC, mg/gdw per year) and R2* (sec-1) for each regime at starting T2* levels of 5, 11 and 17 ms (top panel) and quadratic fit curves respectively (bottom panel) as evaluated from the "repeated measurements analysis".
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 2: Estimated annual rates of change of CIC and R2*. Estimated annual rates of change of CIC (r-CIC, mg/gdw per year) and R2* (sec-1) for each regime at starting T2* levels of 5, 11 and 17 ms (top panel) and quadratic fit curves respectively (bottom panel) as evaluated from the "repeated measurements analysis".
Mentions: Due to the non-linear relationship between T2* and CIC, the estimated constant rate of change of T2* within each severity group corresponds to a variable rate of change for CIC within the same severity group. For this reason, we produced estimates of the annual change of CIC for baseline measurements of 5, 11 and 17 ms (Figure 2, top panel). These values represent, more or less, the middle of each severity range. A quadratic fit curve based on many estimations of r-CIC according to a wide range of values of the baseline T2* (2–18 ms) is also presented in Figure 2 for each treatment regime (bottom panel). The observed increasing trend for r-CIC for baseline T2* values > 15 ms is due to the discontinuous form of iron severity grouping which produced overlapping of the estimated r-CIC between the three iron load groups. This would have been less likely to occur if an extremely large number of patients was available, allowing for a very narrow categorization.

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