A 32-year-old man with sickle cell disease presents with priapism lasting 5 hours. Initial management includes hydration and analgesia. After 4 hours with no resolution (total duration now 9 hours), according to ASH 2020 sickle cell disease guidelines, the next specific intervention is:
- A Exchange transfusion to reduce HbS percentage below 30%
- B Hydroxyurea initiation
- C Intracavernosal aspiration ± phenylephrine injection ✓
- D Sildenafil administration
Explanation
For ischaemic priapism >4 hours, the AUA/ASH guidelines recommend urological intervention: intracavernosal aspiration of blood with or without irrigation, followed by intracavernosal injection of a sympathomimetic agent (phenylephrine is preferred, 100–500 mcg diluted, repeated every 5 minutes for up to 1 hour). Exchange transfusion is considered for refractory priapism not responding to local measures. Hydroxyurea reduces sickle cell crises but is not an acute treatment. Sildenafil (PDE5 inhibitor) is under investigation for prevention but worsens acute priapism by maintaining erection.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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Written and medically reviewed by the StethoPrep medical team.