Abstract

Purpose: Primary intraocular lymphoma is a primary central nervous system lymphoma in which lymphoma cells invade the retina, vitreous, or optic nerve head without concomitant central nervous system involvement. The aim of this presentation is to report a case of primary intraocular non-Hodgkin lymphoma. Methods: The authors present a case report of a 77 years-old female with painless decreased in visual acuity and floaters. Ophthalmic examination, Static computerized Perimetry, Spectral-Domain Optical Coherence Tomography, angiography, laboratory study, lumbar puncture, computed tomography scan and magnetic resonance imaging were performed. Results: On examination, best corrected visual acuity was 20/25 in the right eye and 30/60 in the left eye. Slip lamp examination revealed anterior chamber reaction and fundoscopy showed vitritis, optic disc edema, macular edema and vasculitis in left eye. After three months of follow-up, the patient’s best corrected visual acuity decreased to 20/60 in right eye and light perception in left eye. Fundoscopy revealed vitritis, optic disc edema, macular edema and vasculitis in both eyes. A granular pattern, leakage from retinal vessels and optic disc were observed in fluorescein angiography. Infectious and inflammatory etiologies were excluded. Pars plana vitrectomy and retinal biopsy were performed in left eye. Cytology evaluation revealed atypical lymphoid cells with large nuclei, prominent nucleoli and basophilic cytoplasm, and confirmed the diagnosis of intraocular lymphoma. Conclusion: Intraocular Lymphomas are rare malignancies that display a wide array of clinical manifestation, therefore diagnosis can be challenging. It requires a high degree of clinical suspicion and differential diagnosis includes infectious and non-infectious etiologies.

Highlights

  • Primary Intraocular Lymphoma (PIOL) is a rare malignancy of primary Central Nervous System (CNS) without concomitant intracranial involvement

  • If there was vitreoretinal lymphoma with CNS disease, this is classified as Primary Central Nervous System Lymphoma (PCNSL) [1]

  • On Slit-lamp examination, anterior chamber cells are presented up to 75% of cases

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Summary

Introduction

Primary Intraocular Lymphoma (PIOL) is a rare malignancy of primary Central Nervous System (CNS) without concomitant intracranial involvement. If there was vitreoretinal lymphoma with CNS disease, this is classified as Primary Central Nervous System Lymphoma (PCNSL) [1]. In PIOL, usually, lymphoma cells invade the retina, vitreous, or optic nerve head whereas secondary intraocular lymphoma predominantly infiltrates uveal tract, the choroid. PIOL, usually, presents as a diffuse, large B-cell non-Hodgkin’s lymphoma. The etiology of PIOL and PCNSL are still unknown [1,2]. The average age in immunocompetent patients is the late 50s and 60s and the rate appears to be increasing dramatically, especially in immunocompromised patients [3]

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