Ebstein-Bar Virus (EBV)-Positive diffuse large B-cell vitreoretinal lymphoma: Two cases without overt immunocompromise and review of the literature.

PURPOSE: To describe 2 cases of Epstein-Barr virus (EBV)-positive large B-cell lymphoma of the vitreous and retina (LBCL-VR) in elderly patients without overt systemic immunocompromise and summarize previously published literature.

OBSERVATIONS: Two elderly Asian patients without overt immunocompromise were referred to our clinic for management of uveitis. The first patient, a 79-year-old man, had a history of presumed varicella zoster keratitis of the right eye (OD) and anterior and intermediate uveitis of the left eye (OS). The second patient, a 69-year-old female, had CMV-associated anterior uveitis OD. After initial improvement, both patients developed recurrent and persistent intraocular inflammation leading to a diagnostic vitrectomy. Cytopathology of the vitreous sample confirmed a diagnosis of EBV-positive LBCL-VR in both cases. Neither patient was immunosuppressed nor had evidence of CNS disease. Both patients received serial intravitreal methotrexate as treatment, and both remained free of local recurrence and extraocular dissemination of VRL at last follow up (3 and 1.5 years, respectively).

CONCLUSION

Vitreoretinal lymphoma (VRL) remains a challenging diagnosis. EBV-positive LBCL-VR is an exceedingly rare entity with distinctive demographic and immunologic features. In addition to overt immunocompromise, immunosenescence may also be associated with EBV-positive LBCL-VR. EBV status should be evaluated in all cases of LBCL-VR, especially in LBCL without mutations in MYD88 and/or CD79a.