Ocular manifestations of idiopathic aplastic anemia: retrospective study and literature review

Abstract

Ahmad M Mansour,1 Jong Wook Lee,2 Seung Ah Yahng,2 Kyu Seop Kim,3 Maha Shahin,4 Nelson Hamerschlak,5 Rubens N Belfort,6 Shree K Kurup71Department of Ophthalmology, American University of Beirut, Rafic Hariri University Hospital, Beirut, Lebanon; 2Division of Hematology, Seoul St Mary’s Hospital, The Catholic University of Korea, Seoul, Republic of Korea; 3Department of Ophthalmology, Seoul St Mary’s Hospital, The Catholic University of Korea, Seoul, Republic of Korea; 4Department of Ophthalmology, Mansoura University, Mansoura City, Egypt; 5Oncology and Hematology Program, Instituto Israelita de Ensino e Pesquisa Albert Einstein, São Paulo, Brazil; 6Vision Institute, Hospital São Paulo, Federal University of São Paulo, Brazil; 7Department of Ophthalmology, Wake Forest University Baptist Medical Center, Winston-Salem, NC, USAAbstract: Aplastic anemia (AA) is a rare disease with few reports on its ophthalmic ­manifestations. The ocular findings are described in a retrospective consecutive series of 719 AA Korean patients followed at the Hematology Clinic of The Catholic University of Korea. Out of a total of 719 patients, 269 patients had eye examinations, 156 patients had retinal evaluation, and 37 (23.7%) had retinal findings. These 37 patients had unilateral retinal ­hemorrhage in seven and bilateral retinal hemorrhage in 30 with mean hemoglobin of 6.6 g/dL (range 2.7–12.6 g/dL) and platelet counts of 18.8×109/L (range 4–157×109/L); central retinal vein occlusion-like picture occurred in nine patients and these had similar rheology to the rest of the subjects; optic disc edema, cotton-wool spots, macular edema, and dry eyes occurred in two, three, five, and three patients, respectively. In this Korean series of 141 subjects with AA, systemic bleeding occurred in 24.8% of subjects, retinal hemorrhage in 37% of subjects, and any bleeding site (eye or elsewhere) occurred in 47.5% of subjects with AA. A literature review (1958–2010) of 200 AA cases revealed retinal hemorrhages in 56%, subhyaloid or vitreous hemorrhage in 9%, peripheral retinal vasculopathy in 5.5%, and cotton-wool spots, Sjögren’s syndrome, or optic disc edema in 4% each. The prevalence of retinopathy among series of AA patients varied from 20% to 28.3%, which is consistent with the Korean series of 24.8%. Management of AA patients needs to involve multiple specialties, including hematologists, ophthalmologists, and infectious disease specialists. Keywords: aplastic anemia, eye, retinopathy, retinal hemorrhage, retinal vasculopathy, thrombocytopenia