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Title: Recurrent priapism in Sickle Cell Trait with Protein S deficiency
Authors: Jalil Ur Rehman , Salam S. Al Kindi , Anil V. Pathare , Rajiv Jain , Akram Choudry
Journal: Journal of Pakistan Medical Association
Publisher: Pakistan Medical Association.
Country: Pakistan
Year: 2008
Volume: 58
Issue: 12
Language: English
Abstract
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.
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