近年来日益强大的眼内液分子/细胞生物学检测技术因其简便、快捷和高效的特点,使得眼科医生在诊断眼内淋巴瘤时倾向于单纯只依据此类方法而淡化病理诊断的重要性。眼内液分子/细胞生物学技术因其本身只能“间接提示肿瘤细胞存在”的局限性而不能作为眼内淋巴瘤的确诊依据。眼内组织/细胞病理仍然是眼内淋巴瘤诊断的金标准,其价值和地位不能被其他任何分子/细胞生物学检测手段所替代。理解并掌握各种诊断、检测技术的优势和局限性,规范和优化眼内组织/细胞病理标本的采集、保存和送检流程有助于提高眼科临床医生对眼内淋巴瘤的诊断效率和医疗质量。
In recent years, more and more powerful molecular/cellular biological techniques of intraocular fluid have made ophthalmologists tend to only rely on these methods in the diagnosis of intraocular lymphoma because of their features of simplicity, fastness and efficiency. The molecular/cellular biological techniques of intraocular fluid cannot be used as the basis for the diagnosis of intraocular lymphoma because it can only indicate the existence of tumor cells indirectly. Intraocular tissue/cell pathology remains the gold standard for the diagnosis of intraocular lymphoma, and its importance cannot be replaced by any other molecular/cell biological methods. Understanding and mastering the advantages and limitations of various diagnostic techniques, standardizing and optimizing the collection, preservation and submission process of intraocular tissue/cell specimens will help ophthalmologists improve the diagnostic efficiency and medical quality of intraocular lymphoma.
眼内淋巴瘤(intraocular lymphoma,IOL)比较罕见。按起源位置分为两种类型,主要类型为原发性眼内淋巴瘤(primary intraocular lymphoma,PIOL),也称为原发性中枢神经系统淋巴瘤(primary central nervous system lymphoma,PCNSL);另外一种类型为继发性眼内淋巴瘤(secondary intraocular lymphoma,SIOL),为中枢神经系统以外的淋巴瘤转移至眼内。按肿瘤类型主要分为三类,主要类型为眼内弥漫大B细胞淋巴瘤,属于高级别淋巴瘤,预后较差;其次为少见的主要侵犯脉络膜的黏膜相关淋巴组织结外边缘区B细胞淋巴瘤,属于低级别淋巴瘤,预后较好;第三种类型为极少见的眼内NK/T细胞淋巴瘤,属于高级别淋巴瘤,预后极差。该病的诊断对眼科医生和病理医生都极具挑战性。实验室检测方法主要包括病理学、免疫细胞化学、流式细胞术、细胞因子及基因重排等,但眼内病理活检仍然是该病诊断的金标准。该病的治疗主要为眼内局部化疗、放射治疗及系统性化疗。IOL早期常因误诊而耽误治疗,目前该病明确诊断时多在患者出现症状后4~40个月,多数病例早期被误诊为葡萄膜炎而失去治疗的最佳时机,导致预后较差。因此应充分认识IOL的早期表现,早期诊断、早期治疗,从而大大提高疗效。
Intraocular lymphomas (IOL) are rare malignant neoplasms including primary intraocular lymphoma (PIOL) and secondary intraocular lymphoma (SIOL). The former is also known as primary central nervous system lymphoma(PCNSL). The latter is a kind of lymphoma metastasizing to the eye from outside the central nervous system. IOL can further be divided into three different types. The most common type is vitreoretinal high-grade diffuse large B-cell lymphoma with poor prognosis. The less common type is primary choroidal extranodal marginal zone B-cell lymphoma of mucosa associated lymphoid tissue, which is low-grade B-cell lymphoma with better prognosis.The rare type is NK/T cell lymphomas with very poor prognosis. The diagnosis of this disease is challenging for both ophthalmologists and pathologists. Laboratory testing methods mainly include cytology/pathology,immunocytochemistry, flow cytometry, cytokine analysis and gene rearrangement detection. The detection of malignant lymphoid cells cytologically/pathologically is still the gold standard for diagnosing IOL. The treatment involves local chemotherapy, radiotherapy and systemic chemotherapy. Most intraocular lymphomas at early stage are misdiagnosed as uveitis and proper treatment is often delayed with poor diagnosis due to the lost of best time for treatment. So far, the delay between the diagnosis and the onset of ocular symptoms ranges from 4 to 40 months. Therefore, we should fully understand the early manifestations of intraocular lymphoma and early diagnose and timely treat the disease in order to improve prognosis.