Ocular involvement as first sign of isolated CNS relapse in diffuse large B-cell lymphoma

A 52-year-old man was diagnosed with stage IVB non-Hodgkin lymphoma of diffuse large B-cell lymphoma subtype that involved the bone marrow and liver. Analysis of serum samples showed a raised concentration of lactate dehydrogenase, suggesting a high–intermediate risk of non-Hodgkin lymphoma according to the international prognostic index, but no HIV-1. The patient received six cycles of CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) plus rituximab, and achieved complete remission. 6 months after diagnosis and 2 months after complete remission was achieved, the patient was admitted to hospital because of loss of vision. In the early phase of the examination, a fluorescein angiogram of the right eye (figure A) showed two large hyperfluorescent infiltrates with granularity (arrows) with small regions of retinal pigment epithelial detachments (arrowheads). In the late phase of the study, the left eye (figure B) showed a diffuse retinal pigment epithelial detachment that remained densely hypofluorescent (arrows), suggesting presence of lymphoma. However, bone-marrow biopsy and a CT scan of the neck, chest, and abdomen showed no relapse of lymphoma. 6 days later, the patient became somnolent and agitated, and developed confusion and lethargy. A CT scan (not shown) and an MRI of the brain (figure C) showed multifocal intraparenchymal lesions. Histological and immunohistochemical analysis of a stereotactic biopsy sample of one of the frontal lesions showed presence of B-lymphocyte antigen CD20-positive diffuse large B-cell lymphoma, and no presence of Epstein-Barr virus. The patient was given high-dose methotrexate followed by whole-brain radiotherapy. The patient died 1 month after CNS relapse because of disease progression.