目的：探讨儿童特发性嗜酸性粒细胞增多综合征(IHES)的诊断与治疗。方法回顾性分析1例6岁的IHES患儿的病程及诊治过程。结果患儿以腹部不适起病，短期内出现腹胀、呼吸困难、黄疸、四肢水肿，且肝脾进行性增大。血常规示嗜酸性粒细胞186.39×109/L，骨髓涂片示成熟嗜酸性粒细胞比例明显增加至90.4%，FIL1P1-PDG-FRα融合基因检测、寄生虫、自身抗体检测均为阴性，CT及其他检查提示消化、循环、血液、神经系统均受累。诊断考虑IHES。给予羟基脲联合激素应用，嗜酸性粒细胞数明显下降，但症状、体征无缓解，后出现多脏器功能衰竭。结论儿童IHES临床罕见，治疗、预后有待于进一步探讨。%Objective To discuss the diagnosis and treatment of idiopathic hypereosinophilic syndrome (IHES) in children. Method The course and treatment process of a 6-year-old child with IHES had been retrospectively analyzed. Result The boy was admitted for abdominal discomfort and poor appetite, quickly developed into abdominal distension, dyspnea, jaundice, edema, and worsen hepatosplenomegaly. Routine blood test showed that the eosinophilia was 186.39×109/L. Bone marrow smear showed that the mature eosinophilcell granulocyles signiifcantly increased to 90.4%. The FIL1P1-PDGFRαfusion gene detection, parasites and antibodies tests were all negative. CT and other examinations indicated that the digestion, circulation, blood and nervous system were all affected. The diagnosis of IHES was considered. Hydroxycarbamide and steroids applied, the eosinophil decrease, however, the symptoms no relief, eventually developed to the multiple organ failure. Conclusion IHES is rare in children. Further studies are necessary regarding the treatment and prognosis.
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