Update on the prevalence and pathophysiology of sickle cell priapism: a narrative review.

Priapism is an erectile disorder with persistent and uncontrolled erections without sexual purpose. Ischemic priapism is the most frequent form of priapism and sickle cell disease is a risk factor to develop ischemic priapism with prevalence rates between 30% and 40%. The clear pathogenesis of sickle cell is still under investigation but various mechanisms including nitric oxide, phosphodiesterase type 5, opiorphin and adenosine, testosterone, RhoA/ROCK modulated by oxidative stress and genetic features are known to be key factors of this vascular disease. In this review, we explore these multiple regulatory pathways involved in the pathophysiology of priapism.