Primary Intraocular Lymphoma Associated with Primary Central Nervous System Lymphoma

Purpose: Primary intraocular lymphoma (PIOL) which is considered as a subgroup of primary central nervous system lymphoma (PCNSL) leads to a diagnostic challenge by mimicking various uveitis entities. In this study, we report patients with PIOL in association with central nervous system involvement. Material-Method: We reviewed the medical records of 3 patients diagnosed with PIOL at the Department of Ophthalmology, Istanbul Faculty of Medicine. Results: Two patients were male, one was female. Age at presentation was 69, 32, and 38 years. The initial site of involvement was ocular in one patient and central nervous system in two patients. All of our patients had CD 20-positive large B cell lymphoma diagnosed by stereotactic brain biopsy in two patients and cytologic examination of diagnostic vitrectomy specimen in one patient. Patients received various intravenous, intrathecal chemotherapy and radioterapy treatment regimens. Discussion: PIOL should be considered in the differential diagnosis of uveitis cases of undetermined etiology resistant to standard therapy. All efforts must be made, including invasive diagnostic procedures, in order to diagnose this rare disease.