全面认识眼内淋巴瘤

Comprehensive understanding of intraocular lymphoma

近年来,眼内淋巴瘤的复杂性和多样性逐渐被眼科医生所认知。尽管原发性玻璃体视网膜淋巴瘤是最常见的类型之一,但眼科医生应当意识到其并非唯一的存在,并避免将特定的临床特征过度泛化至所有眼内淋巴瘤类型。眼内淋巴瘤可以根据解剖受累部位分类为玻璃体视网膜淋巴瘤、葡萄膜淋巴瘤(包括脉络膜、虹膜和睫状体淋巴瘤);按病理细胞类型则可分为B细胞、套细胞、T细胞和自然杀伤T细胞淋巴瘤;此外,根据是否伴有眼外组织受累,还可以进一步细分为孤立性眼内淋巴瘤、眼-中枢神经系统淋巴瘤、眼-系统淋巴瘤以及眼-中枢神经系统-系统淋巴瘤。玻璃体视网膜淋巴瘤多见于老年人群,其临床表现类似于葡萄膜炎和白点综合征,对糖皮质激素治疗的反应有限。典型的眼底表现包括玻璃体“薄纱样”或“极光样”混浊以及视网膜下的黄白色肿块。光相干断层扫描在诊断过程中扮演着关键角色,能够揭示视网膜内的垂直强反射以及视网膜内强反射浸润等特异性改变。值得注意的是,玻璃体和视网膜受累的程度可能不同,这对于选择合适的治疗策略具有一定的指导意义。相比之下,葡萄膜淋巴瘤具有独特的临床和病理特征。例如,脉络膜黏膜相关淋巴组织(MALT)淋巴瘤通常表现为慢性病程,而虹膜淋巴瘤中T细胞与B细胞分布大致相等。在诊断方面,脉络膜淋巴瘤通常需要组织病理学检查来确认。而对于MALT淋巴瘤,放射治疗通常是首选治疗方法。T细胞淋巴瘤与B细胞淋巴瘤在眼底表现上相似,但诊断更为复杂,通常需要依赖细胞病理学检查及T细胞受体基因重排检测。对于眼内淋巴瘤患者来说,进行全面系统的排查至关重要,以确定原发部位。对于系统性淋巴瘤患者的眼部病变,还需要进行鉴别诊断,包括考虑肿瘤侵犯、继发性感染以及炎症性病变等因素。随着淋巴瘤发病率的上升,眼科医生应持续更新对眼内淋巴瘤的认识,以确保提供精准的诊断和治疗方案。

In recent years,the complexity of intraocular lymphoma has been gradually recognized by ophthalmologists.Although primary vitreoretinal lymphoma is the dominant type of intraocular lymphoma,ophthalmologists should be aware that it is not unique and avoid overgeneralizing specific clinical features to all intraocular lymphoma types.Intraocular lymphoma can be divided into vitreoretinal,uveal(choroid,iris,ciliary body)lymphoma according to the anatomic affected parts.According to pathological cell types,it can be divided into B cells,mantle cells,T cells and natural killer T cells.At the same time,depending on the presence or absence of extra-ocular tissue involvement,it can also be subdivided into isolated intraocular,oculo-central nervous system,oculo-system,and oculo-central nervous system lymphomas.Vitreoretinal lymphoma tends to occur in the elderly with clinical manifestations similar to uveitis and white spot syndrome and limited response to glucocorticoid therapy.The characteristic fundus manifestations include vitreous gauzy or"auroral"opacity and yellowish-white subretinal mass.Optical coherence tomography plays a key role in diagnosis and can reveal specific changes such as vertical strong reflex and intraretinal strong reflex infiltration.It is worth noting that vitreous and retinal involvement may vary,which has guiding significance for the selection of treatment strategies.In contrast,uveal lymphoma has unique clinical and pathological features,such as the chronic course of choroidal mucosa-associated lymphoid tissue(MALT)lymphoma and the equal distribution of T cells and B cells in iris lymphoma.In diagnosis,choroidal lymphoma often requires histopathological examination,and radiotherapy is the first choice for MALT lymphoma.T-cell lymphoma is similar to B-cell lymphoma in ocular fundus appearance,but diagnosis is more difficult and depends on cytopathology and T-cell receptor gene rearrangement.Comprehensive systematic screening is essential for patients with intraocular lymphoma to identify the primary site.Ocular lesions in patients with systemic lymphoma require differential diagnosis,including tumor invasion,secondary infection,and inflammatory lesions.As the incidence of lymphoma increases,ophthalmologists should constantly update their understanding of intraocular lymphoma to provide accurate diagnosis and treatment.