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目的:了解单克隆IgG沉积的增生性肾小球肾炎临床病理特点。方法:回顾性分析单克隆IgG沉积的增生性肾小球肾炎患者的临床病理资料。结果:9例患者中男性5例,女性4例,肾活检时平均年龄49.8±10.9岁,肾脏病病程15.9±13.1月;血清白蛋白29.0±5.5g/L,8例患者血清白蛋白降低,尿蛋白定量5.6±2.8g/d,7例伴镜下血尿,尿N-乙酰-β-D-氨基葡萄糖苷酶72.3±38.6U/(g·cr),尿视黄醇结合蛋白11.3±17.1mg/L,血清肌酐(SCr)209.5±176.8μmol/L,7例患者SCr升高。补体C3下降5例,补体C4均正常。高血压8例,贫血8例,浆细胞均未见异常,7例行血清免疫固定电泳仅1例存在血清异常IgGκ型条带。肾活检病理光镜为膜增生样病变,3例亦见肾小球结节,1例伴新月体。电镜下见肾小球基膜内皮下、系膜区及少量上皮侧颗粒状电子致密物,其中2例电子致密物呈晶格状结构。患者均有IgG和C3在肾小球内的沉积,5例伴C1q沉积。IgG3κ型3例,IgG3λ型3例,IgG1κ型2例,IgG1λ型1例。结论:单克隆IgG沉积的增生性肾小球肾炎以中老年多见,临床表现大量蛋白尿、血尿,多数患者肾功能不全和贫血,部分出现血清异常单克隆条带。组织学为肾小球膜增生样病变,肾小球见颗粒状电子致密物,少数呈晶格状结构,沉积物以IgG3亚型多见。
Objective: To understand the clinicopathological features of proliferative glomerulonephritis with monoclonal IgG deposition. Methods: The clinicopathological data of patients with proliferative glomerulonephritis deposited by monoclonal IgG were retrospectively analyzed. Results: There were 5 males and 4 females in 9 patients. The average age of renal biopsy was 49.8 ± 10.9 years. The duration of nephropathy was 15.9 ± 13.1 months. Serum albumin was 29.0 ± 5.5 g / L, serum albumin was decreased in 8 cases, Urinary protein was 5.6 ± 2.8g / d, 7 patients with microscopic hematuria, urine N-acetyl-β-D-glucosaminidase 72.3 ± 38.6U / (g · cr), urine retinol binding protein 11.3 ± 17.1 mg / L, serum creatinine (SCr) 209.5 ± 176.8μmol / L, 7 patients SCr increased. Complement C3 decreased in 5 cases, complement C4 were normal. 8 cases of hypertension, 8 cases of anemia, plasma cells were normal, 7 cases of serum immunostaining electrophoresis only 1 case of abnormal serum IgG kappa band. Renal biopsy light microscope for the membrane-like hyperplasia lesions, 3 cases also see glomerular nodules, 1 case with crescent. Electron microscope, see the glomerular basement membrane subendothelial mesangial area and a small amount of epithelial granular electron density, of which 2 cases of electron-dense material was lattice-like structure. Patients had IgG and C3 deposition in glomeruli, and 5 cases had C1q deposition. 3 cases of IgG3κ type, 3 cases of IgG3λ type, 2 cases of IgG1κ type and 1 case of IgG1λ type. Conclusion: The proliferative glomerulonephritis with monoclonal IgG deposition is more common in middle-aged and elderly patients. The clinical manifestations include massive proteinuria, hematuria, renal dysfunction and anemia in most patients, and some abnormal serum monoclonal bands. Histomorphology of mesangial proliferative-like lesions, glomerular see granular electron dense, a few were lattice-like structure, deposition of IgG3 subtype more common.