免疫性全血细胞减少症合并膜增生性肾小球肾炎  

Immunorelated pancytopenia concurrent with membranoproliferative glomerulonephritis with special microtubular deposits

作　　者：胡子云 梁丹丹 曾彩虹 HU Ziyun;LIANG Dandan;ZENG Caihong(National Clinical Research Center of Kidney Diseases,Jinling Hospital,Nanjing Medical University,Nanjing 210016,China)

机构地区：[1]南京医科大学金陵临床医学院(东部战区总医院)国家肾脏疾病临床医学研究中心、全军肾脏病研究所,南京210016

出　　处：《肾脏病与透析肾移植杂志》2023年第3期291-295,共5页Chinese Journal of Nephrology,Dialysis & Transplantation

基　　金：国家自然科学基金面上项目(82070793);江苏省卫健委重点项目(ZD2021018)。

摘　　要：11岁男性患儿,既往有免疫性全血细胞减少症、继发性血友病、脑梗死病史。肾脏损害表现为大量蛋白尿伴少量镜下血尿,肾活检病理提示肾小球膜增生性病变,偶见血栓性微血管病(TMA)样病变,电镜下系膜区和内皮下大量微管状超微结构,直径8~12 nm,最终诊断为肾小球膜增生性病变(考虑与免疫复合物及TMA病变相关)。An 11-year-old boy presented with massive proteinuria and hematuria.The patient had a history of immunorelated pancytopenia,acquired hemophilia and cerebral infarction.Renal biopsy revealed membranoproliferative glomerulonephritis with occasional thrombotic micro-angiopathy-like lesions.On electron microscopy,there were a lot of microtubules in mesangial and subendothelial region,ranging in size from 8 to 12 nm.The final diagnosis was membranoproliferative glomerulonephritis,which was related to immune complex and thrombotic micro-angiopathy lesions.

关 键 词：免疫性全血细胞减少症 膜增生性肾小球肾炎 微管状结构 肾活检 

分 类 号：R725[医药卫生—儿科] R726.9[医药卫生—临床医学]