How is sickle cell disease-associated priapism treated?

Updated: Dec 26, 2019
  • Author: Osama Al-Omar, MD, MBA, FACS, FEBU; Chief Editor: Edward David Kim, MD, FACS  more...
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Answer

Key steps in the management of sickle cell disease–associated priapism include oxygenation, analgesics (eg, intravenous morphine), hydration, alkalization, and exchange transfusions to increase the hematocrit value to greater than 30% and to decrease the hemoglobin S (HbS) value to less than 30%. Although conservative management has commonly been advocated in the literature, several studies have questioned its efficacy, and most experts advocate emergent surgical decompression when conservative management fails.

Ekong and colleagues reported successful use of automated red cell exchange transfusion (ARCET) in five patients with sickle cell disease who were experiencing severely affected by stuttering priapism. Immediately after undergoing ARCET, with a target post-transfusion HbS level below 10%, all five became completely free of stuttering priapism. All five experienced recurrences as their HbS percentage increased towards the end of the ARCET cycle, but with subsequent cycles, most of the patients remained essentially free of stuttering priapism. [33]


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