Graichen D F, Perez E, Jones D B, Font R L
Department of Ophthalmology, Cullen Eye Institute, Baylor College of Medicine, Houston, TX 77030.
Ger J Ophthalmol. 1994 Jan;3(1):54-7.
A 62-year-old man had a 1-year history of bilaterally decreased visual acuity associated with irregular, patchy, grayish-white stromal infiltrates involving both corneas. As visualized by light microscopy, the keratectomy specimen showed numerous homogeneous, eosinophilic deposits located mostly within keratocytes in the posterior one-third of the stroma. The deposits stained reddish-brown with Masson's trichrome but were periodic acid/Schiff-negative. As visualized by electron microscopy, the intracytoplasmic deposits were located within dilated cisternae of rough-surfaced endoplasmic reticulum of keratocytes and disclosed a 10-nm periodicity with a distinct lattice pattern. Immunoperoxidase strains confirmed that the deposits contained immunoglobulin (IgG-kappa). At 2 weeks following a histopathologic examination of the keratectomy specimen, serum protein analysis and immunoelectrophoresis with quantitation of immunoglobulins demonstrated an IgG-kappa monoclonal gammopathy. The abnormal corneal deposits may be the first clinical sign of a malignant lymphoproliferative process that may be associated with a monoclonal gammopathy.
一名62岁男性有1年双侧视力下降病史,伴有累及双眼角膜的不规则、片状、灰白色基质浸润。通过光学显微镜观察,角膜切除术标本显示大量均匀的嗜酸性沉积物,主要位于基质后三分之一的角膜细胞内。这些沉积物用Masson三色染色呈红棕色,但过碘酸/希夫染色阴性。通过电子显微镜观察,细胞质内沉积物位于角膜细胞粗面内质网的扩张池中,呈现出10纳米的周期性和独特的晶格图案。免疫过氧化物酶染色证实沉积物含有免疫球蛋白(IgG-κ)。在对角膜切除术标本进行组织病理学检查2周后,血清蛋白分析和免疫球蛋白定量免疫电泳显示存在IgG-κ单克隆丙种球蛋白病。异常的角膜沉积物可能是与单克隆丙种球蛋白病相关的恶性淋巴增殖性疾病的首个临床征象。