Purpose: Intraocular lymphoma is a rare disease with a poor prognosis. Early diagnosis and early treatment greatly influence the survival prognosis of this disease. This retrospective study aimed to clarify the clinical features of patients diagnosed with intraocular lymphoma, and the diagnostic significance of results from analysis of vitreous samples including cytology, cytokine measurements, and the IgH gene rearrangement test.
Methods: We reviewed 217 patients with intraocular lymphoma diagnosed at 25 medical institutions in Japan. Together with clinical observation, cytological analysis, determination of the levels of cytokines, and/or detection of IgH gene rearrangements were conducted using vitreous fluid specimens. The results were studied in conjunction with clinical findings of intraocular lymphoma. Survival curves were estimated by use of the Kaplan-Meier method.
Results: The subjects comprised 85 men and 132 women, with a mean age at first ophthalmological examination of 63.4 years. The mean observation period was 41.3 months. During the observation period, 69 patients had onset of lymphoma in one eye and 148 had onset in both eyes. Intraocular lymphoma with involvement of the central nervous system (CNS) was most common, found in 60.8 % of the patients, whereas intraocular lymphoma without involvement of other organs was found in 28.1 % of patients. With respect to onset patterns, 82.5 % of patients developed primary ocular lesions whereas 16.1 % developed primary CNS lesions preceding intraocular lymphoma. Blurred vision and ataxia were the most common ocular and extra-ocular symptoms that prompted patients to seek medical examination. Vitreous opacification was the most common ocular finding. The detection rates of malignant cytology, IL-10/IL-6 ratio greater than 1.0, and IgH gene rearrangements in vitreous specimens were 44.5, 91.7, and 80.6 %, respectively, of patients tested. IL-10/IL-6 ratio greater than 1.0 had the highest overall detection rate, and was extremely high (≥90 %) in patients with or without vitreous opacification. The 5-year survival rate was 61.1 %.
Conclusion: Cytokine analysis of vitreous biopsy had the highest detection rate for intraocular lymphoma. This supplementary diagnostic test should be performed frequently to confirm a diagnosis of intraocular lymphoma.