Acute lymphocytic leukemia masquerading as acute retinal necrosis

PURPOSE: To report a case of B-cell acute lymphocytic leukemia (ALL) relapse presenting as acute retinal necrosis. OBSERVATIONS: An 11-year old boy with history of B-cell ALL undergoing maintenance therapy presented with a three-month history of intermittent blurry vision and pain in the right eye when a routine lumbar puncture indicated an elevated lymphoblast-predominant white blood cell count. Bone marrow biopsy revealed 42% lymphoblasts, confirming ALL relapse. Ophthalmic imaging demonstrated a hyperemic optic disc, retinal whitening, perivascular sheathing, retinal hemorrhages, and retinal detachment in the right eye. Vitreous fluid biopsy revealed presence of rare atypical lymphoblasts. Chemotherapy, orbital radiation, and systemic prednisone resulted in improvement of visual acuity and retinal hemorrhages, and resolution of retinal detachment. CONCLUSIONS AND IMPORTANCE: We have described the clinical features, treatment, and response in a case of B-cell ALL relapse with presenting signs of acute retinal necrosis. The uncommon finding in B-cell ALL highlights the possibility of intraocular involvement and the importance of routine ophthalmologic evaluation in leukemia remission.