Noel L H, Droz D, Ganeval D, Grunfeld J P
Clin Nephrol. 1984 May;21(5):263-9.
Eleven cases of renal light chain deposition without amyloïdosis are reported (7 multiple myeloma, one Waldenström's disease, 3 without multiple myeloma without spike in serum or urine). Ten had kappa light chain deposits and 1 lambda light chain deposits along tubular basement membranes and in glomeruli. Ultrastructural study showed granular electron dense material on the external side of tubules with a very dark appearance in 4 cases and lighter appearance in the others. Five cases had nodular glomerulosclerosis with a finely granular, light appearance, corresponding to membrane-like material with kappa fixation in 4. Granular light chain deposition is analogous to type AL amyloïdosis in that their distribution is identical and both originate from light chains. The major difference between AL type amyloïdosis and light chain deposits lies in their ultrastructural appearance. Amyloïd substance is characterized by a fibrillar appearance and light chain substance by a granular appearance.
本文报告了11例无淀粉样变的肾轻链沉积病例（7例多发性骨髓瘤，1例华氏巨球蛋白血症，3例无多发性骨髓瘤且血清或尿液无M蛋白）。10例为κ轻链沉积，1例为λ轻链沉积，沉积于肾小管基底膜和肾小球。超微结构研究显示，4例肾小管外侧有颗粒状电子致密物质，外观极暗，其他病例外观较淡。5例有结节性肾小球硬化，外观呈细颗粒状、淡染，4例对应于κ固定的膜样物质。颗粒状轻链沉积与AL型淀粉样变类似，因为它们的分布相同，且均起源于轻链。AL型淀粉样变与轻链沉积的主要区别在于其超微结构外观。淀粉样物质的特征是纤维状外观，轻链物质的特征是颗粒状外观。
