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

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.