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目的:分析异基因造血干细胞移植术后膜性肾病(MN)的临床病理特征,并探讨其与慢性移植物抗宿主反应(GVHD)之间的关系。方法:选取在我科经活检证实的5例异基因造血干细胞移植(Allo-HSCT)术后MN为研究对象,分析5例患者的临床表现,实验室结果以及肾脏病理形态学、免疫荧光及电镜的病理特点,并行IgG亚型免疫荧光染色,观察不同IgG亚型在肾小球分布的特点及与nephrin分布的关系。此外,利用Western印迹的方法,我们检测了患者血清中是否存在抗M型磷脂酶A2受体(PLA2R)自身抗体。结果:5例患者的临床病理特征如下:(1)移植前无肾脏病史及肾脏病家族史;(2)所有患者在出现蛋白尿的时候均合并存在慢性GVHD(cGVHD)的表现,且4例患者既往有急性GVHD(aGVHD)的病史,经过有效的抗GVHD治疗后,患者的蛋白尿也随之好转;(3)部分患者自身抗体检测阳性,肾组织伴有C4及C1q的沉积,提示体内存在自身免疫现象;(4)肾组织沉积的IgG以IgG4为主,其分布与nephrin一致;(5)抗PLA2R自身抗体的检测结果显示5例患者中仅有1例阳性,阳性率远低于特发性膜性肾病(IMN)的检测结果。结论:我们认为Allo-HSCT术后的MN也是cGVHD的一种表现形式,其发病机制可能与移植入的免疫细胞产生了抗宿主足细胞的抗体有关,且抗体的类型不同于IMN的抗PLA2R自身抗体。
Objective: To analyze the clinicopathological features of membranous nephropathy (MN) after allogeneic hematopoietic stem cell transplantation and to investigate its relationship with chronic graft versus host disease (GVHD). Methods: Five cases of allogeneic hematopoietic stem cell transplantation (Allo-HSCT) confirmed by biopsy in our department were selected as study subjects. The clinical manifestations, laboratory findings and renal pathomorphology, immunofluorescence and electron microscopy The pathological features of IgG were observed by immunofluorescence staining of IgG subcultures. The characteristics of glomerular distribution of different IgG subtypes and the relationship with nephrin distribution were observed. In addition, we examined the presence of anti-M-type phospholipase A2 receptor (PLA2R) autoantibodies in the sera of patients using Western blotting. Results: The clinical and pathological features of the 5 patients were as follows: (1) no history of renal disease and family history of renal disease prior to transplantation; (2) all patients had concurrent manifestations of chronic GVHD (cGVHD) in the presence of proteinuria; and 4 Patients had a history of acute GVHD (aGVHD), and their proteinuria improved after effective anti-GVHD treatment. (3) Positive autoantibodies were detected in some patients, and deposition of C4 and C1q in the kidney tissues suggested that in vivo (4) The IgG in renal tissue was mainly IgG4 and its distribution was consistent with that of nephrin. (5) The results of anti-PLA2R autoantibodies showed that only 1 of 5 patients was positive and the positive rate was much lower than Idiopathic membranous nephropathy (IMN) test results. CONCLUSIONS: We believe that MN after Allo-HSCT is also a manifestation of cGVHD, and its pathogenesis may be related to the production of anti-host podocyte antibodies by the transplanted immune cells, and the type of antibody is different from that of IMN anti-PLA2R itself antibody.