Intraocular Lymphoma Models

Primary vitreoretinal lymphoma (PVRL) is a subtype of primary central nervous system lymphoma (PCNSL), a high-grade, extranodal, non-Hodgkin's lymphoma, predominantly of B-cell origin. PVRL is an aggressive disease with a poor prognosis. Human studies are not ideally suited for the study of intraocular lymphoma pathogenesis or treatment strategies due to the rare nature of the disease, its variable presentation, limited volume of available ocular fluids, and fragility of sampled lymphoma cells. Animal models have been critical in making progress in understanding intraocular lymphoma pathogenesis and investigating potential therapeutic strategies. Early murine models for intraocular lymphoma used intraperitoneal injection of mouse T-cell lymphomas. This was followed by intravitreal T-cell murine models. More recent murine models have used B-cell lymphomas to more closely mimic human disease. The most current B-cell lymphoma models employ a combined approach of inoculating both the mouse vitreous cavity and brain. The challenge in murine models for intraocular lymphoma lies in recreating the clinical features, disease behavior, molecular profile, systemic immunity, and the microenvironment observed in human disease. In the future, animal models will continue to be central to furthering our understanding of the disease and in the investigation of potential treatment targets.