Damato Bertil E, Bever Gregory J, Afshar Armin R, Rubenstein James L
Ocular Oncology Service, Department of Ophthalmology, University of California San Francisco, San Francisco, California, USA.
Division of Hematology/Oncology, Helen Diller Family Comprehensive Cancer Center, University of California San Francisco, San Francisco, California, USA.
Ocul Oncol Pathol. 2019 Jan;5(1):13-19. doi: 10.1159/000487949. Epub 2018 Apr 17.
PURPOSE/BACKGROUND: The aim of this study was to report a patient with vitreoretinal lymphoma with clinical features providing hypothesis-generating insights into the pathophysiology of this disease.
Clinical history and imaging studies (i.e., fundus photography, optical coherence tomography, fundus autofluorescence, and fluorescein angiography) were documented.
A 71-year-old woman presented with a 2-month history of blurred vision in the right eye and bilateral vitreous infiltrates unresponsive to topical and systemic steroids. Vitreous biopsy of the left eye was diagnostic for lymphoma. Bulky subretinal deposits in the right eye responded to systemic therapy. The left fundus showed diffuse hypoautofluorescence and punctate, hyperfluorescent sub-retinal pigment epithelial tumor deposits, which resolved leaving hypoautofluorescent atrophic retinal pigment epithelium (RPE) scars, except inferotemporally, where retinal vasculopathy had occurred.
The clinical features suggest that occlusion of the inferotemporal retinal arteriole prevented sub-RPE lymphomatous deposits and subsequent RPE atrophy in this area of vascular nonperfusion. This suggests that "primary" vitreoretinal lymphoma is secondary to hematogenous spread from systemic loci. This finding, together with the ocular tumor control achieved entirely by systemic therapy, indicates scope for studies investigating systemic treatment protocols, especially those including immune-modulatory agents.
目的/背景：本研究旨在报告一名玻璃体视网膜淋巴瘤患者，其临床特征为该疾病的病理生理学提供了具有启发性的见解。
记录临床病史和影像学检查（即眼底照相、光学相干断层扫描、眼底自发荧光和荧光素血管造影）。
一名71岁女性，右眼视力模糊2个月，双侧玻璃体浸润，局部和全身使用类固醇均无效。左眼玻璃体活检诊断为淋巴瘤。右眼大量视网膜下沉积物对全身治疗有反应。左眼眼底显示弥漫性低自发荧光以及点状、高荧光的视网膜色素上皮下肿瘤沉积物，这些沉积物消退后留下低自发荧光的萎缩性视网膜色素上皮（RPE）瘢痕，但颞下区域除外，该区域发生了视网膜血管病变。
临床特征表明，颞下视网膜小动脉阻塞阻止了RPE下淋巴瘤沉积物的形成以及该血管无灌注区域随后的RPE萎缩。这表明“原发性”玻璃体视网膜淋巴瘤继发于全身部位的血行播散。这一发现，连同完全通过全身治疗实现的眼部肿瘤控制，表明有必要开展研究以探索全身治疗方案，尤其是那些包括免疫调节剂的方案。
