Ophthalmic Manifestations as First Presenting Sign of Evans Syndrome.

Purpose: This work reports a case of Evans syndrome first presenting with ophthalmic signs. Methods: A 27-year-old previously healthy man presented with headaches and bilateral blurred vision for 2 weeks. The visual acuity was 20/30-1 and 20/60-2 in the right eye and left eye, respectively. A fundus examination revealed Roth spots, diffuse multilayer retinal hemorrhages in the macula and periphery, and tortuous vessels in both eyes. Optical coherence tomography showed a disrupted foveal contour from intraretinal fluid and hemorrhage in both eyes. Fluorescein angiography demonstrated dilated and tortuous vessels with scattered blocking defects from hemorrhages. Results: A workup revealed warm hemolytic anemia with severe thrombocytopenia consistent with the diagnosis of Evans syndrome. Conclusions: Evans syndrome is a rare blood dyscrasia that can first present as subacute vision loss and should be added to the differential of diffuse bilateral retinal hemorrhages spanning a multitude of retinal layers.

Highlights on Ocular Toxicity of Immune Checkpoint Inhibitors at a US Tertiary Cancer Center.

Introduction: Immune checkpoint inhibitors (ICIs) have improved prognosis in advanced malignancies; however, they may be associated with extensive ocular immune-related adverse events (irAEs) that are sight threatening. Our study aimed to identify the presentation, characteristics, management, and clinical outcomes of ocular irAEs. Methods: In this retrospective, observational case series, we reviewed the medical records of 1280 patients at a large US tertiary cancer center between 2010 and 2020. Results: We identified 130 patients who presented with ocular irAEs (10%) with 69 males (53%) and 61 females (47%). The mean time to toxicity was 6.1 months. Adverse events include corneal toxicity (31%), neuro-ophthalmic (14%), uveitis and scleritis (13%), retinopathy (13%), periocular disorders (11%), and others. IrAEs occurred most frequently with nivolumab (26%). Most ocular irAEs were treated with topical therapy. Advanced cases required systemic corticosteroids and even cessation of ICIs. Conclusion: Our cohort is a large case series highlighting the increased potential of ocular toxicity associated with ICIs. Prompt recognition and management of ocular irAEs can minimize their effect.