合并嗜酸性粒细胞增多,出现Purtscher样视网膜病变的非典型溶血性尿毒症一例  

Atypical Hemolytic Uremic Syndrome with Eosinophilia and Purtscher-like Retinopathy:A Case Report

作　　者：梅琦敏 戴佳原 刘业成[1,2] 沈敏 朱华栋 MEI Qimin;DAI Jiayuan;LIU Yecheng;SHEN Min;ZHU Huadong(Department of Emergency,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Beijing 100730,China;State Key Laboratory of Complex Severe and Rare Medical,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Beijing 100730,China Diseases;Department of Rare Diseases,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Beijing 100730,China)

机构地区：[1]中国医学科学院北京协和医院急诊科,北京100730 [2]中国医学科学院北京协和医院疑难重症及罕见病国家重点实验室,北京100730 [3]中国医学科学院北京协和医院罕见病医学科,北京100730

出　　处：《协和医学杂志》2025年第1期256-262,共7页Medical Journal of Peking Union Medical College Hospital

基　　金：中国医学科学院临床与转化医学研究专项(2024-12M-C&T-B-014)。

摘　　要：非典型溶血性尿毒症(atypical hemolytic uremic syndrome,aHUS)是一种由补体异常所引发的罕见病,临床表现以微血管病性溶血性贫血、血小板减少症和急性肾损伤为主要特征。本文报道1例以严重肾功能不全伴双眼视力急进性下降起病的患者,病程中出现嗜酸性粒细胞增多症,排除其他疾病后诊断为aHUS。予以激素治疗后嗜酸性粒细胞降至正常,血浆置换联合依库珠单抗治疗后肾脏功能趋于稳定,血小板恢复正常,但视力无明显改善。本文回顾该患者的诊治历程并结合文献复习,以期为临床医师提供借鉴。Atypical hemolytic uremic syndrome(aHUS),a rare disease caused by complement abnormalities,is characterized by microangiopathic hemolytic anemia,thrombocytopenia,and acute kidney injury.In this paper,we report a patient with severe renal insufficiency with rapidly progressive decline in binocular visual acuity,who developed eosinophilia during the course of the disease,and was diagnosed with aHUS after excluding other diseases.After glucocorticoid treatment,eosinophils decreased to normal,and after treatment with plasmapheresis combined with eculizumab,renal function tended to be stable,platelets returned to normal,but visual acuity did not improve significantly.This article reviews the diagnosis and treatment process of this patient and incorporates the review of literature,in the hope of providing reference for clinicians.

关 键 词：非典型溶血尿毒综合征 嗜酸性粒细胞增多症 肾功能不全 视力下降 

分 类 号：R552[医药卫生—血液循环系统疾病] R77[医药卫生—内科学]