【摘要】 目的探讨铁螯合剂治疗高量输血重型β地中海贫血铁过载的临床疗效。方法选择长期维持高量输血的重型β地中海贫血铁过载患者84例,其中不足量使用铁螯合剂患者36例,足量使用铁螯合剂患者48例。不足量使用铁螯合剂患者分为去铁胺(DFO)组、去铁酮(DFP)组、DFO联合DFP组;足量使用铁螯合剂患者分为DFO组、DFP组、地拉罗司(DFX)组、DFO联合DFP组。观察1年后血清铁蛋白(SF)的变化。结果不足量DFO、DFP组,SF均较1年前升高,DFO联合DFP组SF无明显变化;足量DFO、DFP组SF变化差异无统计学意义(P>0.05),DFX组、DFO联合DFP组SF下降差异均有统计学意义(P<0.05),其中DFX组疗效最佳,其次为DFO联合DFP组。结论高量输血重型β地中海贫血患者铁过载的治疗,应长期坚持使用足量的铁螯合剂,DFX以及DFO联合DFP祛铁效果明显。不良反应以骨关节疼痛及胃肠道反应为主。更多还原

【Abstract】 Objective We want to explore the effectiveness of iron chelator to treat the iron overload of βthalassemia major after high-volume blood transfusion.Methods 84 cases of long-term maintenance of high levels of blood transfusion in β-thalassemia major patients were enrolled,36 cases in the insufficient group,and 48 cases in the sufficient group.The insufficient group was divided into 3 subgroups:deferoxamine(DFO),deferiprone(DFP),and DFO combined with DFP.The sufficient group was divided into 4 subgroups:DFO,DFP,DFX and DFO combined DFP.The serum ferritin(SF)changes were observed after one year.Results In the DFO and DPF subgroups of the insufficient group,SF levels were higher than those in a year ago,and in the DFO combined DFP subgroup,SF changes were not significantly different.In insufficient group,the SF changes in the DFO or DPF subgroup were not significantly different.Deferasirox or DFO combined DFP subgroups showed significant decrease in SF levels.The efficacy of Deferasirox is the best,followed by DFO combined with DFP.Conclusion The high-volume blood transfusion therapy for β-thalassemia major iron overload should be treated with sufficient amounts of iron chelator.The effects of Deferasirox and the DFO combined with DFP were good.The most common adverse effects were bone and joint pain and gastroesophageal reaction.更多还原