Cooley Anemia

Cooley Anemia (Thalassemia; Thalassemia Major; Thalassemia Minor)

General: Autosomal dominant in synthesis of the  or  chain of hemoglobin; most prevalent in Mediterranean and Oriental populations.

Ocular: Retinal hemorrhages; angioid streaks; macular vascular abnormalities; pigmented chorioretinal scars (black sunbursts); occlusion of peripheral retinal arteries; vitreous hemorrhages.

Clinical: Hemolytic anemia; hypochromic anemia.

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Goldberg MF. Ophthalmologic manifestations of sickle cell thalassemia. Arch Intern Med 1971; 128:33-39.

O'Donnell BF, et al. Angioid streaks in beta thalassemia minor. Br J Ophthalmol 1991; 75:639,

Rinaldi M, et al. Ocular involvement correlated with age in patients affected by major and intermedia beta-thalassemia treated or not with desferrioxamine. Metab Pediatr Syst Ophthalmol 1993; 16:23-25.