轻链沉积病患者临床病理特征  

Clinico-pathologic features in Chinese patients with light chain deposition disease

作　　者：李晓梅[1] 谌达程[2] 梁丹丹[2] 徐峰[2] 梁少姗[2] 朱小东[2] 张明超[2] 黄湘华[2] 刘志红[2] 曾彩虹[2] 

机构地区：[1]南京大学医学院附属金陵医院(南京军区南京总医院)肾脏科,硕士研究生南京210016 [2]南京大学医学院附属金陵医院(南京军区南京总医院)国家肾脏疾病临床医学研究中心全军肾脏病研究所,南京210016

出　　处：《肾脏病与透析肾移植杂志》2016年第1期1-7,共7页Chinese Journal of Nephrology,Dialysis & Transplantation

基　　金：国家科技支撑计划课题(2013BAI09B04;2015BAI12B05);江苏省临床医学科技专项(BL2012007)

摘　　要：目的:阐明轻链沉积病(LCDD)患者的临床病理特征、预后及其相关危险因素。方法:回顾分析LCDD患者的临床、病理及随访资料,应用Kaplan-Meier法分析肾脏存活率,COX回归模型分析预后危险因素。结果:观察45例LCDD患者,肾活检时平均年龄50.8岁,男∶女1.6∶1。11例(24.4%)患者同时确诊多发性骨髓瘤(MM)。患者高血压、贫血、肾功能不全、尿蛋白定量≥3.5 g/24h和镜下血尿的发生率分别为80.0%、95.6%、95.6%、44.4%和82.2%。血清单克隆免疫球蛋白条带阳性者为26.2%,血清轻链κ/λ>1.65者占84.4%,31.1%为显著异常(>8),33.3%患者补体C3降低。80%的患者组织学改变为肾小球结节样病变,20%为肾小球轻至中度系膜增生,53.3%患者肾小管间质慢性病变重度,77.8%患者光镜下存在轻链相关动脉病变。免疫荧光提示κ轻链沉积占93.3%,沿肾小管和肾小球基膜线样分布,系膜区团块状分布。5例患者失随访,余40例患者平均随访22.1个月,共26例(65.0%)进入ESRD,平均肾脏存活时间为33.8月。单因素和多因素COX回归分析显示,肾活检时血清肌酐(SCr)、尿视黄醇结合蛋白(RBP)是进展至ESRD的重要危险因素,未发现其他临床及病理指标与肾脏预后相关。结论:LCDD多发于中年男性,临床表现高血压、蛋白尿、肾功能不全、贫血,血清轻链比值异常较血清免疫固定电泳是更为敏感的诊断提示。肾小球结节病变、肾小管基膜增厚,电子致密物沿肾小球基膜内侧缘和肾小管基膜外侧缘沉积是主要病理表现。轻链沉积以κ型为主。LCDD患者预后差,SCr、RBP是影响预后的独立危险因素。Objective： To explore the clinico-pathologic features and prognosis in Chinese patients with light chain deposition disease（ LCDD）. Methodology： Forty five patients with LCDD fulfilled the inclusion criteria were recruited this retrospective study. Their clinical,pathologic and follow-up data were collected and analyzed. Kaplan-Meier estimate and Cox regression were used in the analysis of renal survival and prognostic factors respectively. Results： They were 28 males and 17 females with a mean age of 50. 8 years old, and male / female ratio was 1. 6 ∶ 1. 11 patients were simultaneously diagnosed as multiple myeloma（ MM）. In clinical findings,hypertension was presented in 80. 0% of cases,renal insufficiency in 95. 6%, anemia in 95. 6%, nephrotic-range proteinuria（ ≥ 3. 5 g /24h） in 44. 4% and microhematuria in 82. 2%. Monoclonal Ig was detected in 26. 2% of cases by serum immunofixation electrophoresis（ s IFE）. Serum free light chain（ FLC） ratio was abnormal（ κ/λ 1. 65） in 84. 4% of cases,and was markedly abnormal（ κ/λ 8） in 31. 1%. 33. 3% of patients had hypocomplementemia C3. In histopathological observation,80. 0% of patients showed glomerular nodular lesions and the remaining patients（ 20. 0%） displayed mild to moderate mesangial proliferation. Severe tubular atrophy and interstitial fibrosis were found in 53. 3% of patients. 77. 8% of cases had light chain related arteriole lesion on light microscopy. Immunofluorescence revealed deposits of light chain（ 95. 6% κ light chain） linear along the GBMs and TBMs. A total of 40 patients had followed-up for a mean time of 22. 1 months,and 26 patients developed to ESRD. The mean renal survivals were 33. 8 months. Univariate and multivariate COX analysis indicated the serum creatinine（ SCr）（ P = 0. 005） and the level of urinary retinol binding protein（ RBP）（ P〈0. 001） at renal biopsy predicting prognosis. Other clinical and histopathologic parameters did not correlate with renal survival.Conclusion：

关 键 词：轻链沉积病 临床特点 病理特征 预后 

分 类 号：R593[医药卫生—内科学] R692[医药卫生—临床医学]