Chan Chi-Chao, Sen H Nida
Section on Immunopathology, Laboratory of Immunology, National Eye Institute, National Institutes of Health, Bethesda, Maryland 20892, USA.
Discov Med. 2013 Feb;15(81):93-100.
Primary vitreoretinal lymphoma (PVRL), previously called primary intraocular lymphoma (PIOL), is a rare and fatal ocular malignancy. PVRL is a subset of primary central nervous system lymphoma (PCNSL), mostly a diffuse large B-cell lymphoma. The diagnosis of PVRL is often challenging as it often masquerades as chronic uveitis. PVRL requires invasive procedures for tissue diagnosis. Cytology/pathology, molecular pathology (immuno-globulin or T-cell receptor gene rearrangement), immunohistochemistry, biophysical technology (flow cytometry), and cytokine analysis (interleukine-10) are often required. The therapies that have been successful in systemic lymphomas have not been reliably effective in PVRL and PCNSL. Current management of PVRL involves aggressive chemotherapy (methotrexate and rituximab) and radiation therapy. PVRL normally responds well to initial treatment; however, relapse rate and CNS involvement are high, resulting in poor prognosis and limited survival. A professional team of medical experts in ophthalmology, oncology (particularly neuro-oncology), and pathology is essential for optimizing patient management.
原发性玻璃体视网膜淋巴瘤（PVRL），以前称为原发性眼内淋巴瘤（PIOL），是一种罕见且致命的眼部恶性肿瘤。PVRL是原发性中枢神经系统淋巴瘤（PCNSL）的一个亚型，主要是弥漫性大B细胞淋巴瘤。PVRL的诊断通常具有挑战性，因为它常常伪装成慢性葡萄膜炎。PVRL需要通过侵入性检查来进行组织诊断。通常需要进行细胞学/病理学、分子病理学（免疫球蛋白或T细胞受体基因重排）、免疫组织化学、生物物理技术（流式细胞术）和细胞因子分析（白细胞介素-10）。在系统性淋巴瘤中取得成功的治疗方法在PVRL和PCNSL中并未被证实有可靠疗效。PVRL目前的治疗包括积极的化疗（甲氨蝶呤和利妥昔单抗）和放射治疗。PVRL通常对初始治疗反应良好；然而，复发率和中枢神经系统受累率很高，导致预后不良和生存期有限。一个由眼科、肿瘤学（特别是神经肿瘤学）和病理学方面的医学专家组成的专业团队对于优化患者管理至关重要。
