血清抗M型磷脂酶A2受体抗体阳性肿瘤合并膜性肾病6例临床分析

Clinical analysis of 6 cases of anti-phospholipase A2 receptor positive malignancycomp liated with membranous nephropathy

目的探讨血清抗M型磷脂酶A2受体(PLA2R)抗体阳性的肿瘤合并膜性肾病(M-MN)患者的临床特点、治疗方法及预后。方法回顾分析中国医科大学附属盛京医院2016年6月至2019年6月期间诊断的6例M-MN患者的临床资料及治疗方法,并随访患者预后。结果 PLA2R阳性的M-MN患者的肿瘤病理类型与既往研究中出现的肿瘤类型基本符合,4例经肾穿刺活检的患者肾脏病理表现为基底膜不均匀增厚,免疫荧光染色IgG4为阳性,其中1例患者表现为蛋白尿及白蛋白水平表现不一致,2例肿瘤患者存在镜下血尿。6例患者均经手术切除肿瘤,并治疗MN后4例完全缓解,2例未缓解。结论 M-MN肾病有PLA2R相关检测阳性的病例存在,同时临床要重视PLA2R阳性的膜性肾病中有M-MN的可能,以免漏诊误诊,并及时针对性治疗。

Objective To study the clinical symptoms,treatment programs,and prognosis of malignancy-associated membranous nephropathy with positive anti-PLA2 R antibody. Methods Six cases of malignancy-associated membranous nephropathy hospitalized in Shengjing Hospital from Jun.2016 to Jun.2019 were retrospectively studied concerning the clinical information and treatment programs. The patients were followed up and evaluated about the prognosis. Results The tumor pathological types of the M-MN patients with positive PLA2 R are conform to the types reported in previous studies.The pathological manifestations of 4 cases with renal biopsy were uneven thickened basement membrane and IgG4 positivity.One case had hypoalbuminemia but limit proteinuria. Two MN patients with carcinoma had microscopic hematuria. Four cases had complete remission after surgery and therapy of MN,while 2 cases had no remission. Conclusion Malignancyassociated membranous nephropathies may be with positive PLA2 R. In order to avoid missed diagnosis and misdiagnosis,we should pay attention to the possibility of M-MN in positive PLA2 R MN,and give targeted therapy in time.