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一名患有血栓性微血管病并伴有肾小球内皮下电子致密沉积物的患者。

A patient with thrombotic microangiopathy accompanied by glomerular subendothelial electron dense deposits.

作者信息

Kimura M, Fujigaki Y, Ohtake T, Furuya R, Hishida A, Kaneko E

机构信息

University of Shizuoka School of Nursing, Japan.

出版信息

Am J Nephrol. 1998;18(2):155-9. doi: 10.1159/000013326.

Abstract

A 50-year-old woman presented with thrombocytopenia, microangiopathic hemolytic anemia, and nephrotic syndrome. Although a high level of circulating immune complexes, mild hypocomplementemia, and the antinuclear antibody also were present, the criteria for collagen disease were not fulfilled. Renal biopsy demonstrated a typical thrombotic microangiopathy (TMA) involving glomeruli. There also were electron-dense deposits located just beneath the original glomerular basement membrane in the dilated subendothelial space, thought to be immune complexes following positive IgG, C1q and C3 staining on an immunofluorescent study. Corticosteroid therapy rapidly ameliorated her hematologic abnormalities and proteinuria and normalized the immunologic data. These findings strongly suggest that the TMA in this patient was induced by immune complex-associated mechanisms.

摘要

一名50岁女性出现血小板减少、微血管病性溶血性贫血和肾病综合征。尽管存在高水平的循环免疫复合物、轻度补体低下以及抗核抗体,但未满足胶原病的诊断标准。肾活检显示典型的血栓性微血管病(TMA)累及肾小球。在扩张的内皮下间隙中,也有电子致密沉积物位于原始肾小球基底膜下方,免疫荧光研究显示IgG、C1q和C3染色阳性,推测为免疫复合物。皮质类固醇治疗迅速改善了她的血液学异常和蛋白尿,并使免疫数据恢复正常。这些发现强烈提示该患者的TMA是由免疫复合物相关机制诱发的。

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