IgG-γ1型重链1例.DOC

·临床病理讨论· 肾脏重链沉积病（IgG-γ1【摘 要】1例肾脏重链沉积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，血肌酐升至4.96mg/dl时，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）IgG亚型IgG（γ重链单抗）沿肾小球毛细血管袢及肾小管基膜线样沉积，IgA和IgM阴性。IgG2IgG3，IgG4阴性。κ轻链λ轻链染色阴性。电镜下见肾小球基膜内侧及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（Igγ1重链）。 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 Military Command, 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 4.96mg/dl, eGFR 12.9ml/min). 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 revealed high-density deposits inside glomerular basement membrane (GBM) and outside TBM. Based on clinical, laboratory, and histological findings, the patient was finally diagnosed as renal γ1-heavy chain deposition disease.. 【Key words】 heavy chain deposite disease; biopsy; nodular glomerulopathy; Immunoglobulin G; monoclonal immunoglobulin deposition disease Corresponding author: YIN Guang, E-mail: yinguang@ 1 病例摘要 患者，男性，57岁。因“血压升高10年，发现尿检异常、血肌酐升高1年，加重伴浮肿1个月”于2009?07?09入院。 10年前因头晕发现血压高，最高达240/140mmHg，先后服用尼莫地平、尼群地平、北京降压零号等治疗，波动在160/90mmHg左右，当时未做尿检。1年前因肉眼血尿，2d后自行缓解，外院查尿蛋白3+，隐血+，血肌酐（serum creatinine，Scr）1.43mg/dl，血白蛋白34.7g/L并高脂血症，超声双肾不小[左肾（left kidney，LK）/右肾（right kidney，RK）：96mm/96mm]，诊断为慢性肾功能不全，慢性肾炎，高血压。