Hydroxyurea therapy for priapism prevention and erectile function recovery in sickle cell disease: a case report and review of the literature.
Prolonged ischemic priapism in patients with sickling hemoglobinopathies is a urologic emergency requiring immediate intervention to avoid irreversible anoxic penile injury, corporal fibrosis, and erectile dysfunction. Therapeutic options, however, are limited and often ineffective. Here, we report recovery of erectile function with hydroxyurea therapy in an adolescent with hemoglobin SS following a prolonged episode of priapism and subsequent severe erectile dysfunction. This case suggests a potential role of hydroxyurea in reversing end organ damage in patients with hemoglobin SS and also supports basic science work indicating involvement of the NO-dependent pathway in the pathogenesis of sickle cell disease-associated priapism.
Urology, The Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA.
Uzoma A
UA
Anele
Division of Hematology/Oncology and Stem Cell Transplantation, Feinberg School of Medicine, Northwestern University, Chicago, IL 60611, USA.
A Kyle
AK
Mack
Hematology Division, The Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA.
Linda M S
LMS
Resar
Urology, The Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA.
Arthur L
AL
Burnett
D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000150:complications; D000986:Antisickling Agents / Q000627:therapeutic use*; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use*; D008297:Male; D010410:Penile Erection; D011317:Priapism / Q000188:drug therapy* / Q000209:etiology; D020127:Recovery of Function
D000986:Antisickling Agents; D006918:Hydroxyurea