Roth Spots as the Initial Manifestation of Sickle Cell Trait with Suspected Co-inherited Alpha-Thalassemia: A Case Report

Roth spots are white-centered retinal hemorrhages representing a nonspecific morphologic endpoint of retinal capillary rupture. They have long been associated with infective endocarditis, but they are now recognized as a nonspecific sign of systemic microvascular injury. While sickle cell disease is a known cause of retinal vascular complications, their occurrence in sickle cell trait, particularly in association with co-inherited alpha-thalassemia, remains exceptionally rare. This is the case of a previously healthy 40-year-old man presented with sudden visual loss in the left eye, reduced to light perception at initial examination. Fundoscopy revealed multiple Roth spots across all quadrants, extensive macular hemorrhage, and Frisen grade 3 optic disc edema. Fluorescein angiography identified an inferior temporal branch retinal vein occlusion. An exhaustive workup excluded infectious, inflammatory, and malignant etiologies. Peripheral blood smear showed sickled erythrocytes, and hemoglobin electrophoresis demonstrated an HbS fraction of 33.6% with normal HbA2 (3.2%), a pattern consistent with sickle cell trait and suggestive of alpha-thalassemia co-inheritance.This case highlights that Roth spots may be the first ophthalmic sign of an underlying hemoglobinopathy, even in a patient with sickle cell trait. Careful systemic evaluation and multidisciplinary management are essential when white-centered retinal hemorrhages occur without an obvious cause.