Priapism and impotence in homozygous sickle cell disease

Abstract

A questionnaire study of Jamaican patients with homozygous sickle cell (SS) disease indicated a 42% prevalence of priapism, with a median age at onset of 21 yr. Two predominantly different patterns of priapism were recorded: short stuttering episodes lasting < 3 h, with normal consequent sexual function and severe prolonged attacks (generally more than 24 h) commonly followed by impotence. Stuttering episodes were frequently a prodrome to a major attack. Over 1/4 of those who had suffered priapism had some degree of impotence. Hematologic analysis indicated that priapism was significantly associated with low HbF levels and high platelet counts. Patients with severe attacks of priapism had lower HbF levels and reticulocyte count and a higher mean corpuscular volume than patients with only stuttering episodes. Priapism and impotence contribute to the morbidity of SS disease more frequently than previously recognized.