Thorner P S, Bédard Y C, Fernandes B J
Arch Pathol Lab Med. 1983 Dec;107(12):654-7.
Renal disease is a common complication of paraproteinemia, but light-chain nephropathy with resultant Fanconi's syndrome is unusual. In a 60-year-old man with Fanconi's syndrome, the underlying disease was a well-differentiated lymphocytic lymphoma, with two paraproteins and lambda-light-chain proteinuria. A renal biopsy specimen showed crystals in tubular cells, more prominent in proximal than distal tubules. Immunoperoxidase studies confirmed the lambda-light-chain content of the crystals. All previous cases studied by biopsy have involved kappa light chains. Light chains are thought to be toxic to the tubular epithelium, resulting in Fanconi's syndrome. Case reports of lymphoproliferative disorders associated with paraproteinemia and Fanconi's syndrome suggest a somewhat better prognosis with this clinical entity.
肾脏疾病是副蛋白血症的常见并发症，但导致范科尼综合征的轻链肾病并不常见。在一名患有范科尼综合征的60岁男性中，潜在疾病是高分化淋巴细胞淋巴瘤，伴有两种副蛋白和λ轻链蛋白尿。肾活检标本显示肾小管细胞中有晶体，近端小管比远端小管更明显。免疫过氧化物酶研究证实了晶体中的λ轻链成分。此前所有经活检研究的病例都涉及κ轻链。轻链被认为对肾小管上皮有毒性，导致范科尼综合征。与副蛋白血症和范科尼综合征相关的淋巴增殖性疾病的病例报告表明，这种临床实体的预后可能稍好一些。
