Institut national de la santé et de la recherche médicale, UMR S 938, système immunitaire et maladies conformationnelles, Hôpital St Antoine, Paris, France.
Nephrol Dial Transplant. 2011 Sep;26(9):3057-9. doi: 10.1093/ndt/gfr377. Epub 2011 Jul 7.
Kidney involvement with immunoglobulin crystals usually relates to a light chain of the kappa type, in MGUS or smoldering myeloma, frequently causing Fanconi's syndrome with progressive renal insufficiency. We report on a case with severe myeloma featuring lambda light chain-derived crystals and acute kidney injury. Histology showed acute tubular necrosis and tubule obstruction with crystals, which were also abundant inside tubule epithelial cells, macrophages and bone marrow plasma cells. The light chain variable domain had a normal overall primary structure but included 11 somatic mutations, 3 of which likely increased the surface hydrophobicity, as observed in previously reported kappa-type crystals.
肾脏免疫球蛋白晶体沉积通常与单克隆丙种球蛋白血症或冒烟型骨髓瘤中的κ轻链有关，常导致范可尼综合征伴进行性肾功能不全。我们报告一例伴有 λ 轻链衍生晶体和急性肾损伤的严重骨髓瘤病例。组织学显示急性肾小管坏死和晶体管腔阻塞，晶体在肾小管上皮细胞、巨噬细胞和骨髓浆细胞内也很丰富。轻链可变区具有正常的整体一级结构，但包含 11 个体细胞突变，其中 3 个可能增加了表面疏水性，如先前报道的 κ 型晶体所示。
