Limits...
Deferiprone in the treatment of transfusion-dependent thalassemia: a review and perspective.

Galanello R - Ther Clin Risk Manag (2007)

Bottom Line: Comparative studies have shown that at comparable doses deferiprone may be as effective as deferoxamine in removing body iron.Agranulocytosis is the most serious side effect associated with the use of deferiprone, occurring in about 1% of the patients.Combination therapy has been effectively used in the management of severe cardiac siderosis.

View Article: PubMed Central - PubMed

Affiliation: Ospedale Regionale Microcitemie, ASL 8 - Dipartimento di Scienze Biomediche e Biotecnologie, Università degli Studi di Cagliari Italy.

ABSTRACT
Deferiprone is an orally active iron chelator which has emerged from an extensive search for new treatment of iron overload. Comparative studies have shown that at comparable doses deferiprone may be as effective as deferoxamine in removing body iron. Retrospective and prospective studies have shown that deferiprone monotherapy is significantly more effective than deferoxamine in improving myocardial siderosis in thalassemia major. Agranulocytosis is the most serious side effect associated with the use of deferiprone, occurring in about 1% of the patients. More common but less serious side effects are gastrointestinal symptoms, arthralgia, zinc deficiency, and fluctuating transaminases levels. Deferiprone can be used in combination with desferrioxamine. This regimen of chelation is tolerable and attractive for patients unable to comply with standard deferoxamine infusions or with inadequate response to deferiprone alone. Combination therapy has been effectively used in the management of severe cardiac siderosis.

No MeSH data available.


Related in: MedlinePlus

Total iron excretion with different doses of deferoxamine or deferiprone. Red bars indicate mean values. Grey area represents the total iron excretion needed to maintain negative iron balance. Reproduced with permission of RW Grady, Weill Cornell Medical Center.
© Copyright Policy
Related In: Results  -  Collection


getmorefigures.php?uid=PMC2376085&req=5

fig3: Total iron excretion with different doses of deferoxamine or deferiprone. Red bars indicate mean values. Grey area represents the total iron excretion needed to maintain negative iron balance. Reproduced with permission of RW Grady, Weill Cornell Medical Center.

Mentions: The most thorough approach to iron excretion studies has been carried out by Grady et al, who used full iron balance studies to measure iron excreted in both urine and feces and compared the output to the total iron intake with food and blood transfusion. Grady’s DFP and DFO-induced iron excretion results are summarized in Figure 3, and demonstrate a dose-response relationship for DFP between 50 and 100 mg/kg/day and for DFO for 40 and 60 mg/kg/day. Grady’s total iron balance studies in iron overloaded thalassemia patients revealed that 75 mg/kg/day of DFP was comparable to DFO 40 mg/kg/day, and increasing the dose to 60 mg/kg/day of DFO or 100 mg/kg/day of DFP would increase the proportion of patients achieving negative iron balance with these chelators (Grady et al 2001, 2002).


Deferiprone in the treatment of transfusion-dependent thalassemia: a review and perspective.

Galanello R - Ther Clin Risk Manag (2007)

Total iron excretion with different doses of deferoxamine or deferiprone. Red bars indicate mean values. Grey area represents the total iron excretion needed to maintain negative iron balance. Reproduced with permission of RW Grady, Weill Cornell Medical Center.
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC2376085&req=5

fig3: Total iron excretion with different doses of deferoxamine or deferiprone. Red bars indicate mean values. Grey area represents the total iron excretion needed to maintain negative iron balance. Reproduced with permission of RW Grady, Weill Cornell Medical Center.
Mentions: The most thorough approach to iron excretion studies has been carried out by Grady et al, who used full iron balance studies to measure iron excreted in both urine and feces and compared the output to the total iron intake with food and blood transfusion. Grady’s DFP and DFO-induced iron excretion results are summarized in Figure 3, and demonstrate a dose-response relationship for DFP between 50 and 100 mg/kg/day and for DFO for 40 and 60 mg/kg/day. Grady’s total iron balance studies in iron overloaded thalassemia patients revealed that 75 mg/kg/day of DFP was comparable to DFO 40 mg/kg/day, and increasing the dose to 60 mg/kg/day of DFO or 100 mg/kg/day of DFP would increase the proportion of patients achieving negative iron balance with these chelators (Grady et al 2001, 2002).

Bottom Line: Comparative studies have shown that at comparable doses deferiprone may be as effective as deferoxamine in removing body iron.Agranulocytosis is the most serious side effect associated with the use of deferiprone, occurring in about 1% of the patients.Combination therapy has been effectively used in the management of severe cardiac siderosis.

View Article: PubMed Central - PubMed

Affiliation: Ospedale Regionale Microcitemie, ASL 8 - Dipartimento di Scienze Biomediche e Biotecnologie, Università degli Studi di Cagliari Italy.

ABSTRACT
Deferiprone is an orally active iron chelator which has emerged from an extensive search for new treatment of iron overload. Comparative studies have shown that at comparable doses deferiprone may be as effective as deferoxamine in removing body iron. Retrospective and prospective studies have shown that deferiprone monotherapy is significantly more effective than deferoxamine in improving myocardial siderosis in thalassemia major. Agranulocytosis is the most serious side effect associated with the use of deferiprone, occurring in about 1% of the patients. More common but less serious side effects are gastrointestinal symptoms, arthralgia, zinc deficiency, and fluctuating transaminases levels. Deferiprone can be used in combination with desferrioxamine. This regimen of chelation is tolerable and attractive for patients unable to comply with standard deferoxamine infusions or with inadequate response to deferiprone alone. Combination therapy has been effectively used in the management of severe cardiac siderosis.

No MeSH data available.


Related in: MedlinePlus