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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。
Objective To investigate the clinical efficacy of iron chelator in the treatment of iron overload in patients with severe β-thalassemia major transfusion. Methods Eighty-four patients with iron overload of β-thalassemia major who sustained high blood transfusion for a long term were enrolled in this study. Among them, 36 patients were treated with iron chelators insufficiently and 48 patients were given adequate doses of iron chelators. Patients with inadequate iron chelators were divided into DFO group, DFP group, DFO group and DFP group. The patients with sufficient dosage of iron chelator were divided into DFO group, DFP group, DFX) group, DFO combined DFP group. Serum ferritin (SF) was observed after 1 year. The results showed that DFO, DFP group and SF increased compared with that of a year ago, there was no significant change of SF in DFO combined with DFP group. There was no significant difference in SF between full DFO group and DFP group (P> 0.05) There was a significant difference in the descending SF in DFP group (P <0.05), of which DFX group was the best, followed by DFO group and DFP group. Conclusion The treatment of iron overload in high-volume transfusion patients with β-thalassemia major should use enough iron chelators for a long time. DFX and DFO combined with DFP have the obvious effect of removing iron. Adverse reactions to bone and joint pain and gastrointestinal reactions based.