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July 1971

Ophthalmologic Manifestations of Sickle Cell Thalassemia

Author Affiliations

Chicago; Baltimore; Chicago

From the Department of Ophthalmology, University of Illinois Eye and Ear Infirmary, Chicago (Drs. Goldberg and Acacio), and the Department of Medicine, Johns Hopkins University and Hospital, Baltimore (Dr. Charache).

Arch Intern Med. 1971;128(1):33-39. doi:10.1001/archinte.1971.00310190037002

Vascular abnormalities were studied in the eyes of 14 patients with sickle cell thalassemia (S-thal). Angioid streaks were observed for the first time in a patient with S-thal. "Black sunbursts," which are seen more often in the fundi of patients with sickle cell anemia (SS disease) than in those with sickle cell hemoglobin C disease (Hb SC) were generally few in number in the fundi of patients with S-thal. Proliferative sickle retinopathy, which is much more characteristic of Hb SC than of SS disease, was seen in nine patients with S-thal. The retinas of S-thal thus resemble those of Hb SC more than SS. The hemoglobin composition of a patient's red blood cells can be predicted, to a degree, from the presence and type of certain retinal anomalies in sickling disorders. Overall clinical status is not usually as good as indicator of hemoglobin composition.

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