副肿瘤性视网膜病变

定义

副肿瘤性视网膜病变是由源自全身性癌症的肿瘤神经抗原引发的肿瘤免疫反应引起的对视网膜的靶向攻击。患者通常在癌症诊断后出现渐进性视力减退和畏光，但也可能出现视杆（暗适应性受损和周边视力下降）和视锥（视力下降、色彩功能障碍、光敏和眩光）功能障碍。症状往往比临床体征更严重。应排除视网膜病变的其他原因。多种抗视网膜自身抗体（抗恢复蛋白(recoverin)抗体）被描述，尽管其意义尚不确定。两个主要亚组被认可：癌症相关视网膜病变（最常见的是小细胞肺癌）和黑色素瘤相关视网膜病变。 相关的神经自身抗体包括： CRMP5（抗CV2）（坍塌反应调节蛋白5-抗CV2）；抗恢复蛋白抗体；和α-烯醇化酶自身抗体。

Paraneoplastic retinopathies result from a targeted attack on the retina due to a tumour immune response initiated by onco-neural antigens derived from systemic cancer. Patients usually present after cancer diagnosis with progressive visual dimming and photopsias but dysfunction of rods (impaired dark adaption and peripheral vision loss) and cones (decreased visual acuity, colour dysfunction, photosensitivity and glare) may also occur. Symptoms are often worse than clinical signs. Other causes of retinopathy should be excluded. Multiple anti-retinal autoantibodies (e.g. anti-recoverin antibodies) are described although their significance is uncertain. Two major subsets are recognised: cancer-associated retinopathy (most commonly small-cell lung cancer) and melanoma-associated retinopathy. Associated neural autoantibodies include: CRMP5 (anti-CV2) (collapsin response mediator protein 5 - anti CV2); anti-recoverin autoantibodies; and alpha-enolase autoantibodies.

子条目 共 3 条

• 9B71.40黑色素瘤相关的视网膜病变
• 9B71.4Y其他特指的副肿瘤性视网膜病变
• 9B71.4Z未特指的副肿瘤性视网膜病变