HYDROXYUREA IN THE TREATMENT OF SICKLE CELL ASSOCIATED PRIAPISM

Priapism is a sustained, often painful penile erection that is not associated with sexual desire.' It is a common complication of sickle cell disease. Treatment of major episodes of priapism includes initial conservative measures followed by surgical intervention if that has failed aRer 48 hours.2 There is no effective treatment yet for stuttering priapism, although various methods have been used to prevent recurrence without success. Hydroxyurea has been shown recently to be effective in the prevention of sickle cell vaso-occlusive crises.3 We report on a patient with major and stuttering priapism who responded dramatically to treatment with hydroxyurea after several other measures failed. To our knowledge this is the first such case described. CASE REPORT A 25-year-old unmamed Saudi man with sickle cell disease presented to the emergency room in October 1994 with painful persistent penile erection 12 hours in duration. There were no precipitating factors and no associated symptoms. Medical history was significant for several milder episodes during the last 2 months. Examination of the genitalia revealed a rigid erect penis with flaccid glans. Laboratory evaluation demonstrated a white blood count of 8.7 X lo9& hemoglobin 9.8 gm./dl., mean corpuscular volume 79.3 fluid, hemoglobin F 13.9%, hemoglobin S 85.2% and hemoglobin A2 0.9%. He was admitted to the urology ward and started on intravenous fluids. Detumescence spontaneously occurred overnight, and the patient was discharged home. He was readmitted to the hospital in April 1995 for a similar episode of priapism 9 hours in duration. Initial conservative measures with hydration and analgesia failed, and repeated penile aspirations were attempted by the urologist and partial exchange blood transfusion was given. This episode subsided but he continued to have recurrent stuttering priapism. The patient received hydralazine, piracetam and pentoxifylline but the episodes continued to recur during the next 6 months. We decided to include him in our trial of hydroxyurea. A dose of 1,000 mg. hydroxyurea daily was started on September 13,1998 and was increased to 1.5 gm. daily after 4 weeks. He did not report further significant episodes of priapism for 7 months. Hydroxyurea was interrupted for 3 months by the