Priapism in a non-black with sickle cell anemia associated with alpha-thalassemia

Sickle cell anemia, the most prevalent type of hemoglobinopathy, appears almost exclusively in backs. Since the first report of sickle cell anemia in a Caucasian by Cooley and Lee in 1929 [1], additional cases have been reported, mainly from Mediterranean countries, the Persian Gulf, and India [2]. Several cases were also found among Arabs in Israel with a relatively benign clinical course [3, 4]. The present report describes a unique case of sickle cell anemia associated with alpha-thalassemia in a non-black young adult. The diagnosis was made at the age of 23 years. Severe priapism was almost the first manifestation of the disease and subsided following transfusion of five units of packed red blood cells.