【摘要】 目的总结去铁酮(DFP)引起重型β地中海贫血(β-TM)患儿粒细胞缺乏的临床表现,提高对该类副作用的临床认识。方法回顾1例重型β地中海贫血患儿因口服去铁酮引起粒细胞缺乏的相关资料,并进行文献复习。结果女性患儿,7岁1月,因重型β地中海贫血予输血治疗,5周前开始口服去铁酮片除铁治疗,6d前出现反复发热、咳嗽,血常规检查提示粒细胞缺乏,给予抗感染、抗真菌、重组人粒细胞刺激因子等治疗,好转出院。系统检索中国生物医学文献数据库、中国知网、万方数据库、Pubmed、Embase、Web of Science数据库,共8例儿童β-TM因口服DFP致粒细胞缺乏的报道,均为英文文献。9例患儿(含本例)均表现发热,粒细胞缺乏持续1周以上,其中5例发生在服用去铁酮半年内。经积极治疗后均好转出院。结论粒细胞缺乏是去铁酮的严重不良反应,用药前半年内应密切监测粒细胞情况;发现粒细胞缺乏时立即停用去铁酮,并应避免再次使用去铁酮进行除铁治疗。更多还原

【Abstract】 Objective To summarize the clinical manifestations of β-thalassemia major(β-TM)children with agranulocytosis caused by deferiprone(DFP), so as to improve the knowledgement of these side-effects. Methods The clinical data of a month past seven-year-old girl with agranulocytosis due to DFP were summarized and literature was reviewed. Results A girl with transfusion-dependent(3-TM developed agranulocytosis 5 weeks after chelation therapy with DFP. The girl admitted to our hospital due to high fever and cough. Routine blood tests in other hospital revealed that the absolute neutrophil count was 0.05 x 109/L. After hospitalization, anti-infection, anti-fungal, granulocyte colony-stimulation factor(G-CSF) and other treatment were carried out, she was discharged after 12-day-hospital course. A total of 8 cases of children were retrieved from the Chinese biomedical literature database, CNKI, Wanfang,Pubmed, Embase, Web of Science database, all of which were in English literature. The 9 children(including this case) presented with acute high fever, and the duration of agranulocytosis was more than one week. There were 5 cases experienced agranulocytosis within half a year after taking DFP. After active treatment, all of the 9 cases were recovered. Conclusions Agranulocytosis is a serious adverse event of DFP. In the first half year of therapy, neutrophil counts should be monitoring if necessary. Once agranulocytosis has been found, DFP should be stopped and intravenous broad spectrum antibiotic therapy be initiated promptly.更多还原