Priapism in sickle cell disease.

Priapism, an unwanted painful erection of the penis, is a little discussed but common complication of sickle cell disease. What is known about the prevalence of priapism, efficacy of management approaches, and outcome is drawn primarily from retrospective and single-center reports. Priapism occurs in two patterns: prolonged and stuttering (ie, recurrent brief episodes that resolve spontaneously). If priapism persists for 4 hours or more without detumescence, the patient is at risk for irreversible ischemic penile injury, which may terminate in fibrosis and impotence. Large multicenter studies examining the epidemiology and current treatments and well-organized trials of novel therapies are urgently needed for patients who have sickle cell disease and priapism.