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单轻链亚类(κ链)免疫球蛋白在肾小球肾炎中的沉积

Single light chain subclass (kappa chain) immunoglobulin deposition in glomerulonephritis.

作者信息

Alpers C E, Tu W H, Hopper J, Biava C G

出版信息

Hum Pathol. 1985 Mar;16(3):294-304. doi: 10.1016/s0046-8177(85)80017-4.

Abstract

Renal glomerular disease characterized by the deposition of immunoglobulin light chains or monoclonal immunoglobulins was demonstrated by immunofluorescence microscopy in 11 patients. The most common histopathologic findings were those of mesangiocapillary glomerulonephritis, but considerable variability was observed. Lesions resembling diabetic glomerulosclerosis and amyloidosis were seen in some patients. Immunofluorescence findings in seven patients showed concomitant, equally intense staining for kappa light chain and immunoglobulin heavy chain (IgG or IgA), indicative of monoclonal immunoglobulin deposition. Specimens in the remaining cases stained predominantly for kappa light chain alone. In six cases the histologic and ultrastructural pattern was similar to that of type I mesangiocapillary glomerulonephritis. In three cases linear deposits were present, predominantly in subendothelial and inner glomerular basement membranes and, to a lesser degree, in mesangial locations, as in type II mesangiocapillary glomerulonephritis. In one of the latter cases dense deposits were intermixed with aggregates of amorphous fibrillar material indistinguishable from amyloid. In two cases involving IgA kappa chain deposition the histologic and ultrastructural appearance was that of mesangial glomerulonephritis. Considerable heterogeneity was found in the clinical features of the patient population. Specific clinical or serologic parameters for this disease could not be identified. Only one patient had an associated lymphoplasmacytic disorder. After follow-up periods ranging from six months to 17 years, all of the patients were alive, including four who had progressed to end-stage renal disease and required dialysis. Two of the latter patients underwent successful renal transplantation; one had been alive for five years and the other for three months without evidence of recurrence of the renal disease at the last follow-up examination.

摘要

免疫荧光显微镜检查证实,11例患者存在以免疫球蛋白轻链或单克隆免疫球蛋白沉积为特征的肾小球疾病。最常见的组织病理学表现为系膜毛细血管性肾小球肾炎,但也观察到相当大的变异性。部分患者出现类似糖尿病肾小球硬化症和淀粉样变性的病变。7例患者的免疫荧光检查结果显示κ轻链和免疫球蛋白重链(IgG或IgA)同时出现且染色强度相同,提示单克隆免疫球蛋白沉积。其余病例的标本主要仅对κ轻链染色。6例患者的组织学和超微结构模式与I型系膜毛细血管性肾小球肾炎相似。3例患者出现线性沉积物,主要位于内皮下和肾小球基底膜内层,系膜部位也有较少程度的沉积,类似于II型系膜毛细血管性肾小球肾炎。在后一种情况的1例患者中,致密沉积物与无法与淀粉样物质区分的无定形纤维状物质聚集体混合在一起。2例涉及IgA κ链沉积的患者,其组织学和超微结构表现为系膜增生性肾小球肾炎。患者群体的临床特征存在相当大的异质性。无法确定该疾病的特定临床或血清学参数。仅1例患者伴有淋巴浆细胞性疾病。经过6个月至17年不等的随访期后,所有患者均存活,其中4例已进展至终末期肾病并需要透析。后2例患者成功接受了肾移植;1例存活5年,另1例存活3个月,最后一次随访检查时无肾病复发迹象。

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