Coupland Sarah E, Heimann Heinrich, Bechrakis Nikolaos E
Department of Pathology, Charité--University Medicine, Campus Benjamin Franklin, Hindenburgdamm 30, Berlin, 12200, Germany.
Graefes Arch Clin Exp Ophthalmol. 2004 Nov;242(11):901-13. doi: 10.1007/s00417-004-0973-0. Epub 2004 Oct 29.
Primary intraocular lymphoma (PIOL) is a rare non-Hodgkin lymphoma which arises in the retina or the vitreous. It can occur either together with or independently of primary cerebral nervous system lymphoma (PCNSL); the incidence of the latter has significantly increased over the past three decades. PIOL remains one of the most difficult diagnoses to establish, particularly due to its ability to mimic other diseases in the eye and to the limited material which is often available for examination.
The article reviews the clinical, histopathological, molecular biological and biochemical approaches to the diagnosis of PIOL. The differential diagnoses, including other lymphomatous manifestations in the eye, e.g. primary uveal lymphoma, as well as non-neoplastic uveal diseases are addressed. Furthermore, the treatment strategies for PIOL are summarised.
Diagnostic progress has been made in various fields, including flow cytometry and immunocytology, cytokine analysis, and as well as molecular biological analysis of the immunoglobulin heavy and light chains using polymerase chain reaction on both fixed and non-fixed material. The optimal therapy of PIOL remains to be determined: the current trends suggest that combined radiotherapy and chemotherapy, as well as intravitreal chemotherapy, are of value. Novel therapies which may have a role in the future include oral trofosfamide.
Our understanding of the pathogenesis of PIOL/PCNSL remains far from complete. Intensified efforts must be made to determine the cell of origin of PIOL, as well as to establish "molecular signatures", which could be used to decrease diagnostic delay. Further studies, possibly prospective ones, are required to establish the optimal therapy for initial and recurrent disease.
原发性眼内淋巴瘤（PIOL）是一种罕见的非霍奇金淋巴瘤，起源于视网膜或玻璃体。它可与原发性中枢神经系统淋巴瘤（PCNSL）同时发生或独立出现；在过去三十年中，后者的发病率显著增加。PIOL仍然是最难确诊的疾病之一，特别是因为它能够模仿眼部的其他疾病，并且可供检查的材料往往有限。
本文回顾了PIOL诊断的临床、组织病理学、分子生物学和生化方法。讨论了鉴别诊断，包括眼部的其他淋巴瘤表现，如原发性葡萄膜淋巴瘤，以及非肿瘤性葡萄膜疾病。此外，还总结了PIOL的治疗策略。
在各个领域都取得了诊断进展，包括流式细胞术和免疫细胞术、细胞因子分析，以及使用聚合酶链反应对固定和非固定材料进行免疫球蛋白重链和轻链的分子生物学分析。PIOL的最佳治疗方案仍有待确定：目前的趋势表明，放疗和化疗联合以及玻璃体内化疗具有价值。未来可能发挥作用的新疗法包括口服曲磷胺。
我们对PIOL/PCNSL发病机制的理解仍远未完善。必须加大力度确定PIOL的起源细胞，并建立“分子特征”，以减少诊断延迟。需要进一步的研究，可能是前瞻性研究，来确定初发和复发性疾病的最佳治疗方案。
