Steuhl K P, Knorr M, Rohrbach J M, Lisch W, Kaiserling E, Thiel H J
University Eye Clinic, Tübingen, Germany.
Am J Ophthalmol. 1991 Mar 15;111(3):312-8. doi: 10.1016/s0002-9394(14)72315-3.
We treated two patients who had IgG-kappa-light chain monoclonal gammopathy with partially crystalline and partially amorphic corneal deposits. Impairment of vision made keratoplasty necessary for each patient. Histologic examination of the corneal specimens showed deposits that stained positively for Masson's trichrome in all corneal cells. Immunohistochemical tests identified these deposits as IgG-kappa-light chain immunoglobulin fragments. Electron microscopy showed intracellular, rhomboid-shaped deposits enveloped by a membrane. The same deposits appeared in the conjunctival epithelium, within subconjunctival fibrocytes, and in the plasma cells of the bone marrow. Immunoelectrophoresis showed IgG-kappa-light chain fragments in the blood serum, the lacrimal film, and the aqueous humor. This suggests that the intracellular immunoglobulin fragments may have entered the corneal and conjunctival epithelium by way of the lacrimal film, the keratocytes by way of the corneo-scleral limbus vasculature, and the endothelial cells from the aqueous humor.
我们治疗了两名患有IgG-κ轻链单克隆丙种球蛋白病且角膜有部分结晶状和部分无定形沉积物的患者。视力受损使每位患者都需要进行角膜移植手术。对角膜标本进行组织学检查,结果显示在所有角膜细胞中,沉积物对马森三色染色呈阳性。免疫组织化学检测确定这些沉积物为IgG-κ轻链免疫球蛋白片段。电子显微镜显示细胞内有被膜包裹的菱形沉积物。同样的沉积物出现在结膜上皮、结膜下纤维细胞内以及骨髓的浆细胞中。免疫电泳显示血清、泪膜和房水中存在IgG-κ轻链片段。这表明细胞内免疫球蛋白片段可能通过泪膜进入角膜和结膜上皮,通过角膜缘血管系统进入角膜细胞,并从房水进入内皮细胞。