This case report describes a woman in her 60s diagnosed with primary vitreoretinal lymphoma (PVRL) with leptomeningeal involvement. She presented with a 3-month history of bilateral blurred vision. Funduscopy identified autofluorescent leopard spots, and optical coherence tomography demonstrated typical features of PVRL. Diagnosis was confirmed with the detection of MYD88 Leu265Pro variant in ocular fluid and cerebrospinal fluid (CSF). Abnormal lymphoid cells were seen in vitreous biopsy and CSF. There was no evidence of systemic involvement of lymphoma. Treatment consisted of systemic chemotherapy using the MATRix protocol, ASCT along with close clinical follow-up. Due to the rarity of the condition, evidence for the optimal diagnostic approach and management is limited. Therefore, this case provides an insight into our approach, using the recently published European Haematology Association-European Society for Medical Oncology clinical practice guideline for primary central nervous system lymphoma, along with our recommendations on strategies to facilitate earlier diagnosis and treatment.
Thomas et al. (Wed,) studied this question.