不同铁鳌合剂治疗重型β地中海贫血铁过载的临床研究  

A 5-vears clinical trial in iron-overload patients with β-thalassemia major treated by different iron chelators

作　　者：陈娟娟[1] 陈光福[1] 高红英[1] CHEN Juanjuan CHEN Guang）ru GAO Hongying.(Department of Pediatrics, Shenzhen Second People Hospital /The First Affiliated Hospital, Shenzhen University, Shenzhen, Guangdong 518035, China)

机构地区：[1]深圳市第二人民医院、深圳大学第一附属医院儿科,广东518035

出　　处：《中国小儿血液与肿瘤杂志》2017年第4期185-189,222,共6页Journal of China Pediatric Blood and Cancer

摘　　要：目的比较去铁胺(DFO)、去铁酮(DFP)、地拉罗司(DFX)三种不同铁螯合剂治疗重型β地中海贫血(β-TM)铁过载患者5年的临床疗效。方法随机选择规律输血的β-TM铁过载患者31人,按DFO、DFP和DFX分为三个治疗组,分别对血清铁蛋白(SF)、心脏MRIT2*、肝脏MRIT2*值变化进行分析。结果服药5年后,三组SF均明显降低、心脏MRIT2*、肝脏MRIT2*明显升高(P<0.05);组间比较,三组SF水平5年连续监测差异无显著性(P>0.05);心脏MRIT2*组间比较差异无显著性(P>0.05),但与基线相比,第2年DFP组心脏MRIT2*值上升幅度明显高于DFX组、DFO组(P<0.05),后3年差异无显著性;5年连续监测肝脏MRIT2*DFO组高于DFP组、DFX组(P<0.05),与基线相比,DFO组第2年肝脏MRIT2*值上升幅度明显高于DFP组、DFX组(P<0.05),后3年差异无显著性。结论三种铁螯合剂均能有效降低重型β-TM患者SF浓度、去除心脏和肝脏铁沉积;短期内DFP降低心脏铁负荷优于DFO及DFX,DFO降低肝脏铁负荷优于DFP及DFX。Objective To explore effectiveness of different iron chelators （deferoxamine, DFO; deferiprone, DFP; deferasirox, DFX） in 5 years treatment of iron overlord in children with β thalassemia major （β-TM）. Methods Thirty-one β-thalassemia major children with iron overload who received regular blood transfusion were randomly enrolled to three groups. Serum ferritin （SF） , liver MRI T29 and cardial MRI T2 ＊ were detected and compared among the three groups. Results There were no significant differences in the SF level and the cardiac MRI T2 ＊ value among the three treatment groups （ P 〈 0.05 ） in five year＇ s observation. Compared with baseline, the cardiac MRI T2＊ value were higher in DFP than in both of DFO and DFX during the second year （ P 〈 0.05 ） , but no difference for the rest of three years. The liver MRI T2＊ value were higher in DFO group than DFP and DFX groups after 5 years （ P 〈 0.05 ）. Compared with baseline, the liver MRI T2 ＊ value were higher in DFO than in both of DFP and DFX during second year （ P 〈 0.05 ）, but no difference for the rest of three years. Conclusions Three chelators all can effectively reduce SF levels and lower liver and cardiac overload;However, Removing heart iron was significantly effective by DFP versus DFO and DFX in short time; DFO had better effect in removing liver iron overload than DFP and DFX.

关 键 词：重型Β地中海贫血 铁过载 铁螯合剂 磁共振T2＊ 

分 类 号：R725.5[医药卫生—儿科]