ANGIOID STREAKS IN SICKLE-THALASSEMIA

Angioid streaks have been described in a diverse group of diseases inc luding hemoglobinopathies such as sickle cell anemia and beta-thalasse mia. We investigated the prevalence of angioid streaks and pseudoxanth oma elasticum in the rare situation of patients who had compound heter ozygous traits for hemoglobin S and beta-thalassemia. We examined 58 c onsecutive patients with sickle-thalassemia. Of these, 25 were men and 33 were women, and they ranged in age from 19 to 58 years (mean, 32.6 years). Angioid streaks were identified in six of 58 patients (10%), and of these three also displayed the cutaneous lesions of pseudoxanth oma elasticum, which were confirmed by skin biopsy. An expanded study on several relatives of the patients with angioid streaks failed to id entify any similar cases. Statistical evaluation of the main hematolog ic and biochemical parameters in the patients with and without angioid streaks did not demonstrate any significant differences, except that the thalassemic component in all six patients with angioid streaks was beta degrees (that is, did not allow the synthesis of hemoglobin A). We conclude that angioid streaks and pseudoxanthoma elasticum skin les ions occur with an increased frequency in patients with sickle-thalass emia.