Vitreoretinal lymphoma revealing Richter transformation

To report a rare case of Richter syndrome transformation presenting as diffuse large B-cell vitreoretinal lymphoma (DLBCL) in a patient with chronic lymphocytic leukemia (CLL).

A woman in her late fifties with 11q-mutated CLL in remission presented with bilateral, painless blurring of vision. The best-corrected visual acuity was 20/50 in the right eye and 20/80 in the left eye. Fundus examination revealed multiple hypopigmented yellowish subretinal lesions in both eyes. A presumed diagnosis of secondary vitreoretinal lymphoma/leukemic infiltration was made, and multiple intravitreal methotrexate injections were administered until complete regression of lesions and visual recovery. However, a year later, the left eye relapsed with a large placoid, yellowish subretinal infiltration. ^18F-FDG PET-CT scan revealed systemic lymphadenopathy. Subretinal and vitreous biopsy revealed CD20^-and BCL2-positive large atypical lymphoid cells, confirming DLBCL, consistent with Richter transformation in the eye. Although lymph node and tonsillar biopsies showed no systemic involvement, the patient declined prophylactic high-dose methotrexate despite the elevated risk of systemic transformation.

Bilateral vitreoretinal lymphoma presenting as the first manifestation of Richter transformation is exceedingly rare. Early ophthalmic recognition, biopsy, and systemic staging are essential for avoiding diagnostic delays.