Cappellini Maria Domenica, Cohen Alan, Piga Antonio, Bejaoui Mohamed, Perrotta Silverio, Agaoglu Leyla, Aydinok Yesim, Kattamis Antonis, Kilinc Yurdanur, Porter John, Capra Marcello, Galanello Renzo, Fattoum Slaheddine, Drelichman Guillermo, Magnano Carmelo, Verissimo Monica, Athanassiou-Metaxa Miranda, Giardina Patricia, Kourakli-Symeonidis Alexandra, Janka-Schaub Gritta, Coates Thomas, Vermylen Christiane, Olivieri Nancy, Thuret Isabelle, Opitz Herbert, Ressayre-Djaffer Catherine, Marks Peter, Alberti Daniele
Fondazione Ospedale Maggiore Policlinico, Mangiagalli, Regina Elena IRCCS, Universita di Milano, Via F. Sforza 35, 20122 Milan, Italy.
Blood. 2006 May 1;107(9):3455-62. doi: 10.1182/blood-2005-08-3430. Epub 2005 Dec 13.
Deferasirox (ICL670) is a once-daily oral iron chelator developed for the treatment of chronic iron overload from blood transfusions. A comparative phase 3 trial was conducted to demonstrate the efficacy of deferasirox in regularly transfused patients with beta-thalassemia aged 2 years or older. Patients were randomized and received treatment with deferasirox (n = 296) or deferoxamine (n = 290), with dosing of each according to baseline liver iron concentration (LIC). The primary endpoint was maintenance or reduction of LIC; secondary endpoints included safety and tolerability, change in serum ferritin level, and net body iron balance. In both arms, patients with LIC values of 7 mg Fe/g dry weight (dw) or higher had significant and similar dose-dependent reductions in LIC and serum ferritin, and effects on net body iron balance. However, the primary endpoint was not met in the overall population, possibly due to the fact that proportionally lower doses of deferasirox relative to deferoxamine were administered to patients with LIC values less than 7 mg Fe/g dw. The most common adverse events included rash, gastrointestinal disturbances, and mild nonprogressive increases in serum creatinine. No agranulocytosis, arthropathy, or growth failure was associated with deferasirox administration. Deferasirox is a promising once-daily oral therapy for the treatment of transfusional iron overload.
地拉罗司(ICL670)是一种每日服用一次的口服铁螯合剂,用于治疗因输血导致的慢性铁过载。开展了一项3期对照试验,以证明地拉罗司对2岁及以上定期输血的β地中海贫血患者的疗效。患者被随机分组,接受地拉罗司治疗(n = 296)或去铁胺治疗(n = 290),每种药物的剂量根据基线肝脏铁浓度(LIC)确定。主要终点是维持或降低LIC;次要终点包括安全性和耐受性、血清铁蛋白水平的变化以及机体铁净平衡。在两组中,LIC值为7 mg Fe/g干重(dw)或更高的患者,其LIC和血清铁蛋白均有显著且相似的剂量依赖性降低,并且对机体铁净平衡有影响。然而,总体人群未达到主要终点,这可能是因为相对于去铁胺,给予LIC值低于7 mg Fe/g dw的患者的地拉罗司剂量成比例较低。最常见的不良事件包括皮疹、胃肠道不适以及血清肌酐轻度非进行性升高。地拉罗司给药未出现粒细胞缺乏症、关节病或生长发育障碍。地拉罗司是一种有前景的每日一次口服疗法,用于治疗输血性铁过载。