Ophthalmic Manifestations As First Presenting Sign of Evans Syndrome

Overview

Journal J Vitreoretin Dis

Publisher Sage Publications

Date 2023 Apr 3

PMID 37009543

Authors

Anam A Mazharuddin

Ahmad Rehmani

Larry Puthenparambil

John J Alappatt

Wen-Hsiang Lee

Affiliations

Soon will be listed here.

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 and 20/60 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.

References

Hansen D, Moller S, Andersen K, Gaist D, Frederiksen H . Evans syndrome in adults - incidence, prevalence, and survival in a nationwide cohort. Am J Hematol. 2019; 94(10):1081-1090. DOI: 10.1002/ajh.25574. View

Costallat G, Appenzeller S, Costallat L . Evans syndrome and systemic lupus erythematosus: clinical presentation and outcome. Joint Bone Spine. 2011; 79(4):362-4. DOI: 10.1016/j.jbspin.2011.07.004. View

Jaime-Perez J, Aguilar-Calderon P, Salazar-Cavazos L, Gomez-Almaguer D . Evans syndrome: clinical perspectives, biological insights and treatment modalities. J Blood Med. 2018; 9:171-184. PMC: 6190623. DOI: 10.2147/JBM.S176144. View

Ababneh L, Mahmoud I, Al-Rimawi H . A rare case of Evans syndrome associated with sudden loss of vision: A case report. Eur J Ophthalmol. 2018; 30(2):NP1-NP11. DOI: 10.1177/1120672118809579. View

RUBENSTEIN R, YANOFF M, Albert D . Thrombocytopenia, anemia, and retinal hemorrhage. Am J Ophthalmol. 1968; 65(3):435-9. DOI: 10.1016/0002-9394(68)93098-5. View

Zhang J, Chen Y, Yu Z, Liu L . Bilateral Hemorrhagic Retinopathy with Roth Spots in Pediatric-Onset Systemic Lupus Erythematosus and Associated Thrombocytopenia: A Case Report and Review of Literature. Ocul Immunol Inflamm. 2017; 26(8):1150-1153. DOI: 10.1080/09273948.2017.1370652. View

Ling R, James B . White-centred retinal haemorrhages (Roth spots). Postgrad Med J. 1999; 74(876):581-2. PMC: 2361020. DOI: 10.1136/pgmj.74.876.581. View

Zehetner C, Bechrakis N . White centered retinal hemorrhages in vitamin b(12) deficiency anemia. Case Rep Ophthalmol. 2011; 2(2):140-4. PMC: 3214891. DOI: 10.1159/000328123. View

Ohnishi A, Kaneko M, Hori S, Teramura M, Kato S . Vitrectomy for vitreous bleeding and tractional retinal detachment in a case of Evans syndrome. Jpn J Ophthalmol. 2000; 44(2):177-9. DOI: 10.1016/s0021-5155(99)00186-0. View

10.

Simon O, Kuhlmann T, Bittner S, Muller-Tidow C, Weigt J, Wiendl H . Evans syndrome associated with sterile inflammation of the central nervous system: a case report. J Med Case Rep. 2013; 7:262. PMC: 3879039. DOI: 10.1186/1752-1947-7-262. View

11.

Fayyaz A, Igoe A, Kurien B, Danda D, James J, Stafford H . Haematological manifestations of lupus. Lupus Sci Med. 2015; 2(1):e000078. PMC: 4378375. DOI: 10.1136/lupus-2014-000078. View

12.

Carraro M, Rossetti L, Gerli G . Prevalence of retinopathy in patients with anemia or thrombocytopenia. Eur J Haematol. 2002; 67(4):238-44. DOI: 10.1034/j.1600-0609.2001.00539.x. View

13.

DUANE T, Osher R, Green W . White centered hemorrhages: their significance. Ophthalmology. 1980; 87(1):66-9. DOI: 10.1016/s0161-6420(80)35277-9. View

14.

Michel M, Chanet V, Dechartres A, Morin A, Piette J, Cirasino L . The spectrum of Evans syndrome in adults: new insight into the disease based on the analysis of 68 cases. Blood. 2009; 114(15):3167-72. DOI: 10.1182/blood-2009-04-215368. View