Savige J A, Yeung S P, Bierre A R, Kincaid-Smith P
Department of Nephrology, Royal Melbourne Hospital, Melbourne, Australia.
Nephron. 1989;52(2):144-8. doi: 10.1159/000185617.
A 77-year-old man presented with macroscopic haematuria and oligo-anuric renal failure. A renal biopsy showed moderate linear glomerular basement membrane (GBM) staining with lambda light chains alone, but both circulating IgG and lambda light chain activity were present. There was no evidence of a serum or urinary paraprotein and a bone marrow biopsy was normal. Serum lambda light chains did not bind to renal GBM when examined by indirect immunofluorescence. This case suggests that light chains are capable of binding directly to epitopes in the GBM and may initiate injury although by a different mechanism from the IgG anti-GBM molecule.
一名77岁男性患者出现肉眼血尿和少尿性肾衰竭。肾活检显示肾小球基底膜(GBM)仅中度线性染色,仅见λ轻链,但循环IgG和λ轻链活性均存在。无血清或尿副蛋白证据,骨髓活检正常。间接免疫荧光检查时,血清λ轻链不与肾GBM结合。该病例提示轻链能够直接结合GBM中的表位,并可能引发损伤,尽管其机制与IgG抗GBM分子不同。