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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

Deferiprone 3:1 complex with iron and DFP-O-glucuronide
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fig1: Deferiprone 3:1 complex with iron and DFP-O-glucuronide

Mentions: DFP (3-hydroxy-1,2-dimethylpyridin-4-one) is a synthetic analogue of mimosine, an iron chelator isolated from the legume Mimosa paduca (Clarke and Martell 1992). It has 2 pKas, one of 3.6 and the other of 9.9 (Hider and Liu 2003). DFP has strong iron binding properties, with a pFe3+ of 19.6 and a pFe2+ of only 5.6, indicating a high degree of relative specificity for the trivalent form of iron, binding it in a 3:1 complex (Figure 1). Key pharmacologic properties of the compound are shown in Table 1 (Clarke and Martell 1992; Tam et al 2003). As a water-soluble compound having a partition coefficient of 0.11 and with a molecular weight of only 139 Da, it would be expected to move freely through cell membranes throughout the body.


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

Galanello R - Ther Clin Risk Manag (2007)

Deferiprone 3:1 complex with iron and DFP-O-glucuronide
© Copyright Policy
Related In: Results  -  Collection

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

fig1: Deferiprone 3:1 complex with iron and DFP-O-glucuronide
Mentions: DFP (3-hydroxy-1,2-dimethylpyridin-4-one) is a synthetic analogue of mimosine, an iron chelator isolated from the legume Mimosa paduca (Clarke and Martell 1992). It has 2 pKas, one of 3.6 and the other of 9.9 (Hider and Liu 2003). DFP has strong iron binding properties, with a pFe3+ of 19.6 and a pFe2+ of only 5.6, indicating a high degree of relative specificity for the trivalent form of iron, binding it in a 3:1 complex (Figure 1). Key pharmacologic properties of the compound are shown in Table 1 (Clarke and Martell 1992; Tam et al 2003). As a water-soluble compound having a partition coefficient of 0.11 and with a molecular weight of only 139 Da, it would be expected to move freely through cell membranes throughout the body.

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