Hydroxyurea in the management of sickle cell disease-associated priapism: a scoping review

Sickle cell disease (SCD) is an inherited hemolytic-hemoglobinopathy that affects the ability of erythrocytes to deliver oxygen in the body. Hydroxyurea is a pharmacologic agent that has been approved by the Food and Drug Administration (FDA) for the treatment of SCD crises since 1998. It has also been proposed as a therapy for recurrent ischemic priapism (RIP), recurrent prolonged or unwanted erections in the absence of sexual stimulation, which occurs commonly in SCD. The premise behind hydroxyurea's therapeutic effect is that it improves vascular function and hemoglobin metabolism. Its potential role in RIP is to normalize the nitric oxide (NO) signaling pathway, which is fundamental for the regulatory physiology of the erection response. Hydroxyurea has been investigated in diverse research studies, including a recent clinical trial, showing its potential benefit for treating RIP. In this article, we performed a scoping review of the effectiveness of hydroxyurea for the treatment of SCD-associated RIP.