Truong L D, Mawad J, Cagle P, Mattioli C
Department of Pathology, Baylor College of Medicine, Methodist Hospital, Houston, TX 77030.
Arch Pathol Lab Med. 1989 Jul;113(7):781-5.
We report a case of Fanconi's syndrome associated with multiple myeloma, which displayed some unusual features. Although serum immunoelectrophoresis showed no spike, urine electrophoresis revealed monoclonal kappa light chain. The myeloma cells in multiple organs including bone marrow, lymph nodes, spleen, and kidneys were distended with characteristic intracytoplasmic crystals. In the kidneys, identical intracytoplasmic crystals were found in some proximal tubules, distal tubules, collecting ducts, glomerular cells (mostly parietal epithelial and endocapillary cells), and renal interstitial cells. Only monoclonal kappa light-chain protein was demonstrated in these crystals by immunofluorescence and immunoperoxidase techniques, a finding confirmed for the first time (to our knowledge) by immunoelectron microscopic study of the renal biopsy specimens.
我们报告一例与多发性骨髓瘤相关的范科尼综合征,该病例呈现出一些不寻常的特征。尽管血清免疫电泳未显示峰值,但尿液电泳显示出单克隆κ轻链。包括骨髓、淋巴结、脾脏和肾脏在内的多个器官中的骨髓瘤细胞充满了特征性的胞质内晶体。在肾脏中,在一些近端小管、远端小管、集合管、肾小球细胞(主要是壁层上皮细胞和内皮细胞)以及肾间质细胞中发现了相同的胞质内晶体。通过免疫荧光和免疫过氧化物酶技术,在这些晶体中仅证实了单克隆κ轻链蛋白,据我们所知,这一发现首次通过对肾活检标本的免疫电子显微镜研究得到证实。