Ophthalmic Manifestations as First Presenting Sign of Evans Syndrome

Anam A Mazharuddin; Ahmad Rehmani; Larry Puthenparambil; John J Alappatt; Wen-Hsiang Lee

doi:10.1177/24741264211062931

Ophthalmic Manifestations as First Presenting Sign of Evans Syndrome

J Vitreoretin Dis. 2022 Sep 13;6(6):479-484. doi: 10.1177/24741264211062931. eCollection 2022 Nov-Dec.

Authors

Anam A Mazharuddin¹, Ahmad Rehmani¹, Larry Puthenparambil², John J Alappatt³, Wen-Hsiang Lee¹

Affiliations

¹ University of Texas Medical Branch, Galveston, TX, USA.
² Eye Center of Texas, Webster, TX, USA.
³ Houston Retina Associates, Webster, TX, USA.

Abstract

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.

Keywords: Evans syndrome; Roth spots; hemolytic anemia; retinal hemorrhages; thrombocytopenic purpura.

Publication types

Case Reports