单克隆丙种球蛋白病14例骨髓和肾脏活检病理分析

Monoclonal gammopathy:pathological analyses of bone marrow and kidney biopsy in 14 cases

目的探讨单克隆丙种球蛋白病患者骨髓和肾脏活检病理学特征。方法回顾性分析14例单克隆丙种球蛋白病患者的骨髓和肾脏活检病理资料,并复习相关文献。结果14例患者中,2例为多发性骨髓瘤合并轻链管型肾病(light chain cast nephropathy,LCCN):黏稠伴裂纹管型沉积于肾小管伴单核细胞反应、肾小管上皮细胞损伤和肾小管基底膜破裂;1例为意义未明的单克隆丙种球蛋白病(monoclonal gammopathy of unknown significance,MGUS)合并膜性肾病:大量免疫复合物沉积于肾小球上皮下和基底膜内;11例为有肾脏意义的单克隆丙种球蛋白病(monoclonal gammopathy of renal significance,MGRS):包括增生性肾小球肾炎伴单克隆丙种球蛋白沉积(proliferative glomerulonephritis with MIg deposits,PGNMID)2例:肾小球表现为系膜毛细血管增生性肾炎,免疫荧光及电镜显示单克隆丙种球蛋白沉积于系膜区、内皮下,淀粉样变性肾病9例:肾小球系膜区、血管壁呈现均质无定形物质沉积,电镜下可见紊乱、僵硬、8~10 nm淀粉丝沉积于肾小球基底膜、内皮下、系膜区。3例MGRS患者血清和尿液单克隆免疫球蛋白(monoclonal immunoglobulin,MIg)及骨髓穿刺阴性,10例患者肾组织中MIg与血清及尿液检查一致,1例膜性肾病血清及尿液为MIg,而肾内为多克隆免疫球蛋白。结论单克隆丙种球蛋白病异质性高,肾脏病理结合骨髓病理检查可提高诊断率,有利于单克隆丙种球蛋白病的临床诊治。

Purpose To investigate the pathological characteristics of bone marrow and kidney biopsy in patients with monoclonal gammaglobulin disease.Methods Fourteen cases of monoclonal gammopathy were diagnosed in the Department of Nephrology of this hospitalt,and the pathologic features of kidney biopsy and bone marrow biopsy were analyzed.Results Among the 14 patients,2 had multiple myeloma with light chain cast nephropathy(LCCN):viscous cast deposited in renal tubules with monocyte reaction,injury of renal tubular epithelial cells and rupture of renal tubular basement membrane were noted.1 had monoclonal gammopathy of unknown significance(MGUS)with membranous nephropathy in which immune complexes were deposited in the subepithelium and basement membrane.11 had monoclonal gammopathy of renal significance(MGRS),including 2 cases of proliferative glomerulonephritis with MIg deposits(PGNMID)in which membranoproliferative glomerulonephritis with deposition of monoclonal immunoglobulin in the mesangial area and subendothelium was noted and 9 cases of renal amyloidosis showed that homogeneous amorphous material was deposited in mesangial area and vascular wall,and diam-eter 8-10 nm amyloid substance were detected by electron microscopy.Serum/urine MIg and bone marrow biopsy in 3 patients with MGRS was negative.In 10 patients,the MIg in the kidney was consistent with the serum/urine examination.One case of membranous nephropathy had MIg in the serum/urine and polyclonal immunoglobulin in the kidney.Conclusion Monoclonal gammopathy has high heterogeneity.Kidney and bone marrow pathological examination increases the detective rate,which is beneficial to the clinical diagnosis and treatment of monoclonal gammopathy.