目的:观察肿瘤相关膜性肾病(MN)的临床病理特点,并探讨肿瘤与MN的关系. 方法:回顾分析临床与病理诊断为MN的患者10例,均在肾活检同时或1年内确诊为恶性肿瘤,观察其临床病理特点及转归.检测肾活检时血清抗M型磷脂酶A2受体(PLA2R)自身抗体,并进行肾小球IgG亚型免疫荧光染色. 结果:10例患者中男性9例,女性1例.起病年龄40~65岁,平均年龄(53.6±6.67)岁.导致MN的恶性肿瘤均为实体瘤,其中肺癌(5例)、胃癌(2例)及结肠癌、舌癌,喉癌各1例.肾活检时仅3例患者分别有咯血、呕血及大便潜血阳性等肿瘤相关症状,余7例患者在常规检查中或随访中发现肿瘤.肾脏主要临床表现为蛋白尿和水肿,其中8例表现为肾病综合征(NS),肾功能均正常.光镜下部分患者可见少量单个核细胞和中性粒细胞浸润,无明显肾小管间质急性损伤.10例中3例患者抗 PLA2R自身抗体阳性;8例患者肾组织以IgG1沉积为主(IgG4阴性),2例抗 PLA2R自身抗体阳性患者肾组织IgG1和IgG4沉积强度相同.随访中,7例抗 PLA2R抗体阴性患者中2例肿瘤切除术后蛋白尿转阴,2例未能行手术NS持续,3例因肿瘤恶化死亡.3例抗 PLA2R抗体阳性患者均行手术切除肿瘤,1例术后蛋白尿始终未好转,2例手术后蛋白尿曾转阴,但半年后复发. 结论:导致MN的恶性肿瘤主要来自上皮细胞的肿瘤,其临床表现及病理特点与特发性膜性肾病(IMN)并无明显差异.多数恶性肿瘤相关性MN患者血清抗PLA2R抗体阴性,肾组织以IgG1沉积为主提示其发病机制不同于IMN.而少数患者血清中存在抗PLA2R抗体,肾组织IgG1、IgG4均有沉积提示可能为IMN合并肿瘤.%Objective:To study the clinical and pathologic features of membranous nephropathy (MN) patients associated with tumor and to explore the relationship between MN and tumor. Methodology: Ten cases of tumor associated MN were evaluated retrospectively. The anti-PLA2R (M-type phospholipase A2 receptor) autoantibodies in serum were detected and the glomerular IgG subclass deposits were observed. Results:10 cases were diagnosed as cancer of various types, including carcinomas of lung (5 cases), stomach (2 cases), colon ( 1 case), larynx ( 1 case), and tongue (1 case). All of those patients presented proteinuria and edema; 8 patients showed nephrotic syndrome. The serum Anti-PLA2R autoantibodies were detected in 3 cases (30％), and lgGl deposits was the dominant IgG on the glomeruli in 8 cases (80％). However, the glomerular IF intensities of IgG4 deposits were the same as lgG1 in 2 cases with AntiPLA2R autoantibodies. During follow-up, in seven patients without Anti-PLA2R autoantibodies, the complete remission of the proteinuria was observed in 2 patients associated with tumor remission; 3 patients died of cancer; the other 2 patients had persistent proteinuria. While in three patients with Anti-PLA2R autoantibodies, they had persistent or relapse of proteinuria. Conclusion :The clinical and pathologic features of tumor-associated MN were not significantly different from idiopathic MN. Most of patients had no Anti-PLA2R autoantibodies and had glomerular IgG1 deposits, which indicated that the pathogenesis of tumor-associated MN differs from that of idiopathic MN. The patient with Anti-PLA2R autoantibodies and glomerular IgG4 deposits may suggest the coincidence of tumor and idiopathic MN.
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