Zhou Min, Xu Gezhi
Department of Ophthalmology, Fudan University Eye and ENT Hospital, Fudan University, Shanghai, PR China.
Shanghai Key Laboratory of Visual Impairment and Restoration, Fudan University, Shanghai, PR China.
Taiwan J Ophthalmol. 2016 Oct-Dec;6(4):170-176. doi: 10.1016/j.tjo.2016.05.002. Epub 2016 Jun 27.
Primary vitreoretinal lymphoma (PVRL) was previously termed primary intraocular lymphoma. PVRL is a potentially fatal intraocular malignancy, and 65-90% of PVRL cases eventually involve the central nervous system (CNS). The incidence of PVRL has been rising in both immunocompromised and immuno-competent populations worldwide. PVRL frequently masquerades as chronic uveitis. Advanced auxiliary examinations, such as optical coherence tomography and fundus autofluorescence have been applied in the diagnosis of PVRL. Histology and immunohistochemistry in combination with molecular tests and interleukin-10 analysis have been demonstrated as reliable in diagnosing PVRL. Despite early initiation of treatment, mortality is high with PVRL associated with CNS involvement and relapses are common. The use of systemic chemotherapy has not been proven to prevent CNS involvement; however, local therapies including intravitreal injections of methotrexate and/or rituximab and low-dose radiotherapy to the eye, has shown to be extremely effective in controlling intraocular lymphoma with encouraging results.
原发性玻璃体视网膜淋巴瘤（PVRL）以前被称为原发性眼内淋巴瘤。PVRL是一种潜在致命的眼内恶性肿瘤，65%至90%的PVRL病例最终会累及中枢神经系统（CNS）。在全球免疫功能低下和免疫功能正常的人群中，PVRL的发病率一直在上升。PVRL常伪装成慢性葡萄膜炎。先进的辅助检查，如光学相干断层扫描和眼底自发荧光已应用于PVRL的诊断。组织学和免疫组化结合分子检测和白细胞介素-10分析已被证明在诊断PVRL方面可靠。尽管早期开始治疗，但与CNS受累相关的PVRL死亡率很高且复发常见。全身化疗尚未被证明可预防CNS受累；然而，包括玻璃体内注射甲氨蝶呤和/或利妥昔单抗以及眼部低剂量放疗在内的局部治疗，已显示在控制眼内淋巴瘤方面极其有效且结果令人鼓舞。
