Priapism as an Initial Presentation of Sickle Cell Disease: A Case Report

Priapism is a urological emergency defined as an erection lasting more than four hours unrelated to sexual stimulation. Most cases are ischemic and often related to hematologic disorders, especially sickle cell disease (SCD). About 30 to 40 percent of males with SCD experience priapism, usually starting in adolescence. The pathophysiology is linked to nitric oxide depletion, dysregulation of phosphodiesterase type 5, oxidative stress, and genetic factors. Although common, priapism is often overlooked and undertreated, which can lead to erectile dysfunction and psychological problems. A 21-year-old African American man with no prior medical history presented to Henry Ford St. John Hospital with a painful erection of four days' duration. Laboratory evaluation revealed hemoglobin of 11.5 g/dL (reference range: 13.5-17.5 g/dL) and a positive sickle cell solubility test. Hemoglobin electrophoresis confirmed SCD (HbS 61.9%, HbF 35.9%). Initial interventions, including cavernosal aspiration and phenylephrine injection, were unsuccessful, necessitating distal cavernosal shunt surgery. Hydroxyurea therapy was initiated, and the patient was discharged. Four days later, the patient presented a recurrence of priapism. Symptoms resolved after red blood cell exchange transfusion. He was discharged on hydroxyurea and sildenafil, with multidisciplinary follow-up arranged. This case shows that patients without a prior SCD diagnosis can present with priapism as the first sign, even without typical lab abnormalities. Early recognition and prompt treatment, including urologic procedures, hydroxyurea, and red blood cell exchange transfusion, are important to prevent recurrence and long-term complications. Collaboration among urology, hematology, and mental health specialists is crucial for the care of this patient population.