Probst L E, Hoffman E, Cherian M G, Yang J, Feagan B, Adams P, Nichols B
Department of Ophthalmology, University Hospital, University of Western Ontario, London, Canada.
Cornea. 1996 Jan;15(1):94-8.
The deposition of copper on Descemet's membrane and the anterior and posterior lens capsule with extreme hypercupremia and IgG hypergammaglobulinemia has been previously described with multiple myeloma and pulmonary carcinoma. A 66-year-old man presenting with blurred vision was found to have bilateral golden-brown metallic dust-like deposits on the central region of Descemet's membrane and the anterior and posterior lens capsule. Laboratory investigations revealed an elevated serum copper level 10 times the normal level associated with a monoclonal gammopathy and a normal ceruloplasmin level. Copper binding to the serum proteins was investigated by three biochemical methods. The results demonstrated that the major copper binding fraction in the serum was IgG. N-terminal amino acid analysis of the IgG did not find the sequence of Asp-Ala-His, which has been shown to be a copper binding site in albumin. This is the first report of benign monoclonal gammopathy being associated with the ocular deposition of copper.
先前在多发性骨髓瘤和肺癌患者中曾有过关于极度高铜血症和高IgG血症时铜在Descemet膜以及晶状体前、后囊膜沉积的描述。一名66岁视力模糊的男性患者被发现其Descemet膜中央区域以及晶状体前、后囊膜上有双侧金棕色金属粉尘样沉积物。实验室检查显示血清铜水平升高至正常水平的10倍，伴有单克隆丙种球蛋白病，而铜蓝蛋白水平正常。采用三种生化方法研究了铜与血清蛋白的结合情况。结果表明血清中主要的铜结合成分是IgG。对IgG进行N端氨基酸分析未发现Asp-Ala-His序列，该序列已被证明是白蛋白中的铜结合位点。这是关于良性单克隆丙种球蛋白病与眼部铜沉积相关的首次报道。
