title: Daily labile plasma iron as an indicator of chelator activity in Thalassaemia major patients.
abstract: Labile plasma iron (LPI), a non-transferrin-bound component of plasma iron detected in iron overload disorders is a potential source of cellular iron accumulation and ensuing oxidative damage. Periodic monitoring of LPI over a 24 h time-span was used to compare the ability of chelation to control daily LPI levels in 40 Thalassaemia major patients (9-11/group) who had been receiving one of three different chelation protocols for more than a year: Group I. deferrioxamine overnight, Group II. deferiprone daily, Group III. deferrioxamine and deferiprone sequentially. An additional group (Group IV) was treated with desferasirox for up to 6 months. The patterns of daily LPI recrudescence showed significant individual variations, especially in patients treated with deferrioxamine or deferiprone, although these patterns were maintained over 6-9 months of treatment in all groups. Group data analysis showed that the proportion of patients whose daily LPI were maintained within the normal range (<0.45 micromol/l) varied with treatment: 6/10 with deferrioxamine, 5/11 with deferiprone, 9/10 with deferrioxamine + deferiprone and 8/10 at the onset and 10/10 after 6 months treatment with deferasirox. Although the clinical significance and therapeutic value of LPI remain to be established, monitoring of daily LPI level may provide an analytical basis for assessing chelation efficacy in preventing daily LPI recrudescence.
authors.affiliation: Unita' Day Hospital Talassemici, Ospedale Sant' Eugenio, Rome, Italy.
authors.forename: Giuliana
authors.initials: G
authors.lastname: Zanninelli
authors.forename: William
authors.initials: W
authors.lastname: Breuer
authors.forename: Zvi I
authors.initials: ZI
authors.lastname: Cabantchik
mesh_terms: D000328:Adult; D015415:Biomarkers / Q000097:blood; D002940:Circadian Rhythm; D000077543:Deferiprone; D003676:Deferoxamine / Q000627:therapeutic use; D016903:Drug Monitoring / Q000379:methods; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D007501:Iron / Q000097:blood*; D007502:Iron Chelating Agents / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D011728:Pyridones / Q000627:therapeutic use; D017086:beta-Thalassemia / Q000097:blood / Q000188:drug therapy*
chemical_list: D015415:Biomarkers; D007502:Iron Chelating Agents; D011728:Pyridones; D000077543:Deferiprone; D007501:Iron; D003676:Deferoxamine