文档介绍：·临床病理讨论·
肾脏重链沉积病(IgG-γ1型重链)1例
尹广*,吴燕,曾彩虹,陈惠萍
(南京军区南京总医院全军肾脏病研究所,南京 210016)
【摘要】讨论1例肾脏重链沉积病(IgG-γ1重链)。57岁男性,临床表现高血压、肾病综合征、大量蛋白尿、肾功能不全,病情进行性发展,,双肾超声体积仍不小。肾外损害不明显。肾活检光镜下肾小球呈“结节样病变”,刚果红染色阴性。免疫荧光见免疫球蛋白G(IgG)和IgG亚型IgG1(γ1重链单抗)沿肾小球毛细血管袢及肾小管基膜(TBM)线样沉积,IgA和IgM阴性。IgG2,IgG3,IgG4阴性。κ轻链和λ轻链染色阴性。电镜下见肾小球基膜内侧及TBM外侧电子致密物沉积。通过临床-实验室-病理结合,光镜-免疫病理-电镜结合,特别是针对性免疫病理检查,确诊肾脏重链沉积病(IgG-γ1重链)。
【关键词】重链沉积病;活组织检查;肾小球结节样病变;免疫球蛋白G;单克隆免疫球蛋白沉积病
【中图分类号】 R692 【文献标识码】 A 【DOI】
Renal monoclonal immunoglobulin heavy chain deposite disease (IgG-γ1 heavy chain)
YIN Guang*, WU Yan, ZENG Cai-Hong, CHEN Hui-Ping
(Institute of Nephrology, Nanjing General Hospital, Nanjing mand, Nanjing 210016, China)
【Abstract】 A case of renal heavy chain deposition disease (IgG-γ1 heavy chain) was discussed. A 57-year-old male presented with hypertension, massive proteinuria, microscopic hematuria, and progressive renal dysfunction (serum creatinine , eGFR ). However, ultrasound examination showed that bilateral kidneys were within normal range in size. In terms of histologic examination, nodular glomerulopathy was identified under light microscopy with negative Congo-staining result. Immunofluorescent study showed IgG and IgG1 deposit along glomerular tufts and tubular basement membrane (TBM). Meanwhile, IgA, IgM, IgG2, IgG3, IgG4 and light chain staining was absent. Electron microscopy reveale