Milam A H, Saari J C, Jacobson S G, Lubinski W P, Feun L G, Alexander K R
Department of Ophthalmology, University of Washington, Seattle 98195-0001.
Invest Ophthalmol Vis Sci. 1993 Jan;34(1):91-100.
This study's goal was to determine the pathophysiology of the retinopathy that occurs in patients with metastatic cutaneous melanoma and sudden onset of night blindness, the so-called melanoma-associated retinopathy (MAR) syndrome. We tested the hypothesis that sera from two MAR patients contained autoantibodies that reacted with "on" bipolar cells of the human retina.
Immunofluorescence was performed on cryostat sections of unfixed normal human retinas. Sera and IgG fractions were tested from the two MAR patients and 38 control subjects (28 patients with metastatic melanoma, but no visual symptoms; two patients with non-MAR retinopathy; and eight normal subjects).
The sera and IgG fractions from both MAR patients but from none of the control subjects produced heavy immunostaining of bipolar cells, which were identified as rod bipolars by a double labeling procedure using anti-protein kinase C.
We hypothesize that MAR patients generate autoantibodies against a melanoma antigen that cross react with bipolar cells of the retina. These antibodies, by an unknown mechanism, may cause abnormalities of the rod and cone systems that are characteristic of MAR.
本研究的目的是确定转移性皮肤黑色素瘤患者中出现的视网膜病变以及突然发生的夜盲症(即所谓的黑色素瘤相关性视网膜病变(MAR)综合征)的病理生理学机制。我们检验了这样一个假设,即两名MAR患者的血清中含有与人类视网膜“开启型”双极细胞发生反应的自身抗体。
对未固定的正常人视网膜冰冻切片进行免疫荧光检测。检测了两名MAR患者以及38名对照受试者(28名转移性黑色素瘤患者,但无视觉症状;两名非MAR视网膜病变患者;以及八名正常受试者)的血清和IgG组分。
两名MAR患者的血清和IgG组分对双极细胞产生了强烈的免疫染色,但对照受试者的血清和IgG组分均未产生这种染色。通过使用抗蛋白激酶C的双重标记程序,这些双极细胞被鉴定为视杆双极细胞。
我们推测MAR患者产生了针对黑色素瘤抗原的自身抗体,该抗原与视网膜双极细胞发生交叉反应。这些抗体通过未知机制可能导致MAR特有的视杆和视锥系统异常。