眼内-中枢神经系统淋巴瘤：临床特征、诊断及预后

Intraocular-central nervous system lymphoma: clinical features, diagnosis, and outcomes.

作者信息

Akpek E K, Ahmed I, Hochberg F H, Soheilian M, Dryja T P, Jakobiec F A, Foster C S

机构信息

Ocular Immunology Service, Massachusetts Eye and Ear Infirmary, Boston 02114, USA.

出版信息

Ophthalmology. 1999 Sep;106(9):1805-10. doi: 10.1016/S0161-6420(99)90341-X.

DOI:10.1016/S0161-6420(99)90341-X

PMID:10485554

Abstract

OBJECTIVE

To analyze the clinical features, laboratory investigations, and diagnosis of intraocular-central nervous system (CNS) lymphoma in a cohort of patients who underwent diagnostic vitrectomy.

DESIGN

Retrospective case series. METHOD AND STUDY MATERIALS: Thirty-four vitreous biopsy specimens obtained from 26 patients with treatment-resistant or unusual uveitis were re-evaluated in a masked fashion. The specimens were classified into three groups: "negative," "suspicious of malignancy," and "positive" based on the cytologic features, immunomarkers, and flow cytometry. The medical records of the patients were reviewed retrospectively.

MAIN OUTCOME MEASURES

The reliability of vitreous cytology in diagnosing intraocular-CNS lymphoma and the differences in clinical features of patients with intraocular-CNS lymphoma and uveitis.

RESULTS

The two ocular pathologists concurred in their criteria for interpretation of all specimens. There was 100% concordance between the cytologic reports read independently by the two ocular pathologists over the 5-year period and the read-out done in a masked fashion at the time of the study. Ten patients were diagnosed with intraocular-CNS lymphoma based on the vitreous cytology and clinical features. The time interval between the initial presentation and vitreous biopsy was 1 week to 2 years, with 80% of the patients diagnosed within the first year. Retinal involvement in the form of lymphomatous subretinal pigment epithelial infiltrates, vasculitis, and apparent retinochoroiditis was present in six cases. Initial neuroimaging studies revealed concomitant CNS involvement in three patients, and an additional three developed CNS lymphoma following diagnosis by vitreous biopsy. Patients were treated with radiotherapy, chemotherapy, or both. Two of the four patients with a follow-up of greater than 12 months died due to CNS involvement.

CONCLUSIONS

Vitreous cytology is a sensitive, reliable, and reproducible method of diagnosing intraocular-CNS lymphoma. A high index of suspicion based on the clinical findings and course of the uveitis is critically important in decision-making for diagnostic vitrectomy. Central nervous system involvement is frequent and associated with a high mortality rate. Ophthalmology 1999;106:1805-1810

摘要

目的

分析一组接受诊断性玻璃体切割术患者的眼内 - 中枢神经系统（CNS）淋巴瘤的临床特征、实验室检查及诊断情况。

设计

回顾性病例系列研究。方法与研究材料：对从26例难治性或不典型葡萄膜炎患者获取的34份玻璃体活检标本进行盲法重新评估。根据细胞学特征、免疫标志物及流式细胞术，将标本分为三组：“阴性”、“可疑恶性”和“阳性”。对患者的病历进行回顾性分析。

主要观察指标

玻璃体细胞学诊断眼内 - CNS淋巴瘤的可靠性，以及眼内 - CNS淋巴瘤患者与葡萄膜炎患者临床特征的差异。

结果

两位眼科病理学家对所有标本的解读标准一致。在5年期间，两位眼科病理学家独立阅读的细胞学报告与研究时盲法读出的结果之间的一致性为100%。基于玻璃体细胞学和临床特征，10例患者被诊断为眼内 - CNS淋巴瘤。初次就诊至玻璃体活检的时间间隔为1周至2年，80%的患者在第一年内被诊断。6例患者存在以淋巴瘤性视网膜色素上皮下浸润、血管炎和明显视网膜脉络膜炎形式的视网膜受累。初次神经影像学检查显示3例患者伴有中枢神经系统受累，另外3例在玻璃体活检诊断后发生中枢神经系统淋巴瘤。患者接受了放疗、化疗或两者联合治疗。4例随访时间超过12个月的患者中有两例因中枢神经系统受累死亡。