Matsuyama N, Joh K, Yamaguchi Y, Aizawa S, Kanai T, Kitajima T, Sakai O
Department of Pathology, Jikei University School of Medicine, Tokyo, Japan.
Am J Kidney Dis. 1994 Jun;23(6):859-65. doi: 10.1016/s0272-6386(12)80141-7.
We report a case of a 40-year-old woman with benign monoclonal gammopathy who developed focal segmental glomerulosclerosis associated with widespread deposition of large crystalline inclusions in the glomerular podocytes. Electron microscopy showed that the crystalline structures were electron dense and needle shaped or polygonal. At a higher magnification, they were seen to be surrounded by unit membranes and to feature a longitudinal filamentous substructure. These inclusions showed positive immunohistochemical staining for kappa light chain. Clinically, the patient had shown nephrotic syndrome on the first medical examination, and later revealed chronic renal failure and monoclonal gammopathy of the immunoglobulin G kappa type, but no evidence of multiple myeloma or other lymphoproliferative diseases. This patient seems to have had a rare form of glomerular involvement associated with benign monoclonal gammopathy.
我们报告一例40岁患有良性单克隆丙种球蛋白病的女性，该患者发生局灶节段性肾小球硬化，伴有肾小球足细胞中大量结晶性包涵体的广泛沉积。电子显微镜显示，这些晶体结构电子密度高，呈针状或多边形。在更高放大倍数下，可见它们被单位膜包围，并具有纵向丝状亚结构。这些包涵体κ轻链免疫组化染色呈阳性。临床上，该患者首次体检时表现为肾病综合征，后来出现慢性肾衰竭和免疫球蛋白G κ型单克隆丙种球蛋白病，但无多发性骨髓瘤或其他淋巴增殖性疾病的证据。该患者似乎患有与良性单克隆丙种球蛋白病相关的一种罕见的肾小球受累形式。
