Bilateral Combined Retinal Vascular Occlusion: An Ocular Presentation in Central Nervous System Re-relapse of Non-Hodgkins Lymphoma

Aim: We describe an atypical case of central nervous system (CNS) involvement in non-Hodgkins lymphoma (NHL), presenting with ocular manifestations.

Presentation: Here we present an extremely rare case of a 63 year old male with past history of relapsed NHL in remission with R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone) chemotherapy, primarily presenting with bilateral sequential acute loss of vision without any systemic manifestations of relapse. On ophthalmological evaluation, he was found to have mixed central retinal artery and central retinal vein obstruction (CRAO & CRVO). CSF (cerebro-spinal fluid) evaluation revealed large B cell lymphoma cells. Management was initiated with triple intrathecal chemotherapy.

Discussion: NHL is the commonest type of ocular lymphoma, which is often the first manifestation of systemic relapse in cases of previous therapy. However, CRVO secondary to NHL is extremely rare. To the best of our knowledge, there are no previous reports of bilateral CRVO as a result of re-relapse of DLBCL presenting with features of ophthalmoplegia.

Conclusion: Re-relapse as isolated CNS lymphoma at second remission in a patient with systemic NHL is quite rare. Although unlikely, bilateral combined CRAO & CRVO may be the first manifestation of relapsed CNS lymphoma. Hence, clinicians should have a raised index of suspicion in cases of NHL presenting primarily with sudden, total loss of vision.