Zou W, Wang S, Zhang Y
Department of Pathology, Beijing Medical University.
Zhonghua Bing Li Xue Za Zhi. 1995 Jun;24(3):146-8.
Five patients with glomerulopathy and glomerular fibrillary protein deposits similar to those of amyloid but lacking the Congo red tinctorial affinity characterizing amyloid were studied. Clinically, these patients had severe proteinuria and microscopic hematuria, in addition, 2 patients had hypertension and renal function impairment. 5 renal biopsies were evaluated by light microscopy (LM), immunofluorescence (IF), immunoperoxidase (IP) and electron microscopy (EM). LM: mesangioproliferative, membranous (late stage) and membranoproliferative (late stage) patterns were seen in 2, 1 and 2 patients respectively. IF & IP: granular deposits, mainly of IgG, C3 and kappa, lambda light chains were found in all cases, either in mesangium and/or capillary walls. EM: randomly distributed fibrils with a diameter approximately 21nm (the diameter of amyloid fibrils is about 9.5nm) were found in glomerular basement membrane and/or mesangium. The pathologic findings suggested a new clinicopathological entity differentiated from amyloidosis. The relatively homogeneous nature of the immunoglobulin in the immune deposits is the basis for the fibril formation.
对5例患有肾小球病且肾小球有纤维状蛋白沉积的患者进行了研究，这些沉积类似于淀粉样蛋白，但缺乏淀粉样蛋白特有的刚果红染色亲和力。临床上，这些患者有严重蛋白尿和镜下血尿，此外，2例患者有高血压和肾功能损害。对5份肾活检标本进行了光镜（LM）、免疫荧光（IF）、免疫过氧化物酶（IP）和电子显微镜（EM）评估。光镜：2例、1例和2例患者分别出现系膜增生性、膜性（晚期）和膜增生性（晚期）病变模式。免疫荧光和免疫过氧化物酶：所有病例均在系膜和/或毛细血管壁发现颗粒状沉积物，主要为IgG、C3以及κ、λ轻链。电镜：在肾小球基底膜和/或系膜中发现直径约21nm的随机分布的原纤维（淀粉样原纤维直径约为9.5nm）。病理结果提示这是一种有别于淀粉样变性的新的临床病理实体。免疫沉积物中免疫球蛋白相对均一的性质是原纤维形成的基础。
