Angioid streaks in thalassemia intermedia: warning for thromboembolic events

Pseudoxanthoma elasticum-like lesions have been described in association with an increased risk for stroke in β-thalassemia. We describe a patient with thalassemia intermedia and angioid streaks who developed a central retinal artery occlusion and subsequent portal vein thrombosis.

Case report of a 47-year-old woman with thalassemia intermedia, angioid streaks, central retinal artery occlusion, and portal vein thrombosis.

Our patient presented with a cilioretinal artery-sparing central retinal artery occlusion in the left eye and evidence of angioid streaks in both eyes. One month later, she developed neovascularization of the angle in the left eye and underwent panretinal photocoagulation. Neovascularization of the angle resolved, and final vision was 20/30. She later developed portal vein thrombosis, and anticoagulation was started.

The finding of angioid streaks in the setting of thalassemia may suggest the presence of a systemically defective elastin layer. This can be a procoagulative factor and may warrant further assessment of the coagulation and cardiovascular system to take prophylactic measures. The occurrence of a thromboembolic event, however, is multifactorial and the end result of interaction of procoagulant and anticoagulant factors.