Haematologica · 2011 · 43 citations · 16 references
MedicineHematologyPharmacologyClinical PharmacologyHepatic PharmacotherapyIron Metabolism DisordersAnemia Of Chronic DiseaseDeferoxamine TherapyIron Chelation TherapyIron Deficiency DisordersIron HomeostasisBody Iron StoresCardiac Outcome AssessmentDeferoxamine MonotherapyLabile Plasma IronLiver Iron Concentration AssessmentLiver Iron Concentration MeasurementSingle-arm Trial EvidenceSingle-arm Trials
The US04 trial was a multicenter, open-label, single arm trial of deferasirox monotherapy (30-40 mg/kg/day) for 18 months. Cardiac iron response was bimodal with improvements observed in patients with mild to moderate initial somatic iron stores; relationship of cardiac response to labile plasma iron is now presented. Labile plasma iron was measured at baseline, six months, and 12 months. In patients having a favorable cardiac response at 18 months, initial labile plasma iron was elevated in only 31% of patients at baseline and no patient at six or 12 months. Cardiac non-responders had elevated labile plasma iron in 50% of patients at baseline, 50% patients at six months, and 38% of patients at 12 months. Risk of abnormal labile plasma iron and cardiac response increased with initial liver iron concentration. Persistently increased labile plasma iron predicts cardiac non-response to deferasirox but labile plasma iron suppression does not guarantee favorable cardiac outcome. Study registered at www.clinicaltrials.gov (NCT00447694).
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Survival in Medically Treated Patients with Homozygous β-Thalassemia
Nancy F. Olivieri, David G. Nathan, James H. MacMillan et al. · New England Journal of Medicine · 1994 · 864 citations · Full text
Medicine, Cardiology, Hematology +15
Labile plasma iron in iron overload: redox activity and susceptibility to chelation
Breno Pannia Espósito, William Breuer, Pornpan Sirankapracha et al. · Blood · 2003 · 476 citations · Full text
Medicine, Biochemistry, Hematology +16