Recurrent priapism in sickle cell trait with protein S deficiency.
We report the case of a young Omani man, a regular blood donor, who presented twice in two months, with painful penile erection lasting more than 12 hours. The patient is known to have sickle cell trait [HbS 34.6%]. Although the first episode of penile erection settled with aspiration of blood and local injection of epinephrine, on the second occasion necessitated cavernosal glandular shunting. A subsequent investigation revealed a mild protein S deficiency. Although priapism is known to occur in sickle cell disease, it is unusual in sickle cell trait. Association of mild protein S deficiency with erythrocytosis could have precipitated the onset of priapism.
Rehman
Jalil Ur
JU
Department of Haematology, Sultan Qaboos University Hospital, Sultanate of Oman.
Kindi
Salam S Al
SS
Pathare
Anil V
AV
Jain
Rajiv
R
Choudry
Akram
A
D000328:Adult; D006801:Humans; D008297:Male; D011317:Priapism / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D018455:Protein S Deficiency / Q000150:complications* / Q000503:physiopathology; D012008:Recurrence; D012805:Sickle Cell Trait / Q000150:complications* / Q000503:physiopathology