Retinal vasculitis as a presenting sign of Epstein-Barr virus positive diffuse large B-cell lymphoma: a case report

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BACKGROUND: To present a case of bilateral retinal vasculitis associated with diffuse large B-cell lymphoma (DLBCL). CASE PRESENTATION: A 28-year-old Asian male presented with irritation, a foreign body sensation, and blurry vision in his left eye. While in India, he experienced back pain and a left-sided neck nodule, which was assessed via ultrasound and considered idiopathic. Ocular examination revealed peripheral vascular sheathing bilaterally. Fluorescein angiography demonstrated small areas of hyperfluorescence around the nasal foveal vessels, mild vasculitis in the right eye, and prominent vasculitis in the left eye. A physical exam identified lymphadenopathy in the cervical chain. Therefore, a chest CT was performed which showed possible lymphoproliferative/metastatic disease or granulomatous infection followed by PET-CT demonstrating splenic, bone marrow, and extralymphatic organ involvement. An initial core biopsy of the left supraclavicular lymph node was performed and revealed rare, atypical large CD30 + lymphoid cells suggestive of Non-Hodgkin lymphoma, along with CD20 + large, atypical B-cells. A subsequent excisional biopsy confirmed Epstein-Barr Virus (EBV)+ DLBCL, stage IV. Following chemotherapy, retinal vasculitis resolved, and vision remained stable. CONCLUSIONS: Although rare, retinal vasculitis can serve as an early manifestation of EBV+ DLBCL. Its diagnosis is particularly challenging due to its ability to mimic other conditions. However, maintaining a high index of suspicion, comprehensive physical exam, and initiating aggressive treatment are essential.