BACKGROUND AND OBJECTIVE: To describe the incidence, characteristics, and risk factors of a pigmentary maculopathy in patients with primary central nervous system (CNS) lymphoma treated with blood-brain barrier disruption (BBBD) therapy. PATIENTS AND METHODS: This retrospective chart review included patients with biopsy-proven primary CNS lymphoma treated with or without BBBD therapy who underwent an ophthalmic examination after starting systemic treatment. Clinical data and all available retinal imaging were analyzed. RESULTS: Fifty-eight patients met inclusion criteria. Twenty-one of 36 patients treated with BBBD therapy had a bilateral pigmentary maculopathy. None of the 22 patients treated with conventional chemotherapy had similar changes. Additional findings in patients treated with BBBD included geographic retinal pigment epithelium atrophy, subretinal fluid, and in one case, choroidal neovascularization. Some cases of maculopathy resulted in reduced visual acuity. The presence of a pigmentary maculopathy was associated with a higher number of BBBD treatment sessions (20.1 vs 13.3, P =.016), but not vitreoretinal lymphoma involvement or intravitreal methotrexate injections. CONCLUSION: In this cohort, 58.3% of patients with primary CNS lymphoma treated with BBBD and chemotherapy were found to have a bilateral pigmentary maculopathy. This maculopathy can result in reduced visual acuity and is associated with the number of BBBD treatment sessions.
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