Follow‐up of sickle cell disease patients with priapism treated by hydroxyurea

Abstract

Hydroxyurea is one of the most successfully used therapies for sickle cell disease. Results of many clinical trials point to hydroxyurea administration for patients with frequent painful crises and acute chest syndrome. Priapism is one of the complications that could be prevented by hydroxyurea, but there are few reports demonstrating the results. Since November 1993, hydroxyurea has been used in our clinic for preventing priapism in patients with stuttering or major attacks who are still capable of achieving intercourse on demand. Five patients were enrolled in the study, and 4 cases benefited by this treatment. After the initial treatment for the acute attack, all five patients developed stuttering priapism. Hydroxyurea was then introduced at the initial dose of 10 mg/kg, and as the hydroxyurea dosage increased, the number or length of priapism episodes decreased. One to two months after the maximal dose (20-35 mg/kg) was introduced, the episodes disappeared. In two patients, we were forced to administer over 30 mg hydroxyurea/kg to abort the episodes, and, in another patient, 25 mg/kg was necessary. All patients present normal sexual activity. Hydroxyurea was discontinued in two patients, but stuttering priapism reappeared. Hydroxyurea was then re-introduced, and priapism disappeared. One patient, using 20 mg hydroxyurea/kg, had a 6-year remission of priapism after hydroxyurea administration; however, he experienced stuttering priapism, 1 month before a major attack, that progressed to impotence. During that month, he did not seek medical attention. In conclusion, the data here presented suggests that hydroxyurea may prevent priapism attacks in sickle cell disease, probably at higher doses than usually prescribed for painful crisis prevention. Am.

Medical and surgical management of priapism

“…50 Five patients were enrolled in the study, and four cases benefited by this treatment. After the initial treatment for the acute episode, all five patients developed stuttering priapism.…”

Hemolysis-associated priapism in sickle cell disease

“…Five patients with stuttering priapism, whose average age was 20 years, experienced relief during hydroxyurea treatment. 13 Their average increase in hemoglobin was 16 g/L (1.6 g/dL) while HbF increased by 8.6%. Hydroxyurea, which increases hemoglobin concentration in HbSS by increasing HbF concentration and reducing hemolysis, 54 is also a NO donor and may directly activate cellular guanylate cyclase, increasing cGMP.…”

“…3 Since then, many researchers have estimated its incidence and prevalence in various populations and the choices for its treatment. [2][3][4][5][6][7][8][9][10][11][12][13][14][15][16] In general, about 30% of males with SCD under the age of 20 years reported having had at least one episode of priapism, 8,11,17 whereas frequencies of 30% to 45% are estimated for adult men. 18 Among patients with priapism, about 75% had their first occurrence before the age of 20 with the mean age being around 12 to 15 years.…”

Recent Advances in the Medical and Surgical Treatment of Priapism

“…Other medical treatments including estrogens, hydroxyurea, gabapentin, tissue plasminogen activator, methylene blue, and digoxin have been reported to play a role in the prevention of priapism [28][29][30][31][32][33][34]. Nonetheless, evidence for their clinical use is limited.…”