CAJ | 학술논문

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。
목적：탐토인동특발성기산성립세포증다종합정(IHES)적진단여치료。방법회고성분석1례6세적IHES환인적병정급진치과정。결과환인이복부불괄기병，단기내출현복창、호흡곤난、황달、사지수종，차간비진행성증대。혈상규시기산성립세포186.39×109/L，골수도편시성숙기산성립세포비례명현증가지90.4%，FIL1P1-PDG-FRα융합기인검측、기생충、자신항체검측균위음성，CT급기타검사제시소화、순배、혈액、신경계통균수루。진단고필IHES。급여간기뇨연합격소응용，기산성립세포수명현하강，단증상、체정무완해，후출현다장기공능쇠갈。결론인동IHES림상한견，치료、예후유대우진일보탐토。
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.