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Introduction: Priapism is a rare and true urological emergency characterized by persistent penile erection that continues at least 4 hours without sexual stimulation. Hematological disorders are the second common cause of priapism. Among patients with leukemia, 50% of leukemic priapism was due to chronic myeloid leukemia (CML). However, as a presenting feature of CML, priapism is rare (1-2%). Herein, we share a case of a patient who presented with priapism as the first manifestation of CML.
Case: A 27-year-old gentleman, with no known medical illness presented with 24 hours of painful penile erection. No history of trauma, sexual intercourse, use of illicit drugs or medication. He had significant constitutional symptoms for the past 3 months. His brother passed away at young age from leukemia. He was pale, not jaundiced and his vital signs were within normal limits. Abdominal examination revealed hepatosplenomegaly. Perineum examination showed engorged, tender and erected penis. Blood investigation showed white blood cells 756x103/uL, hemoglobin 7g/dL and platelet 142x103/uL. Cavernous blood gas showed, pO2 21mmHg, pCO2 100mmHg, pH 7.10. Urgent peripheral blood film was suggestive of CML with blast cells 7%. Patient was given hydration and oral hydroxyurea. Therapeutic aspiration from corpus cavernosum was done and priapism resolved. However, priapism recurred hence he was referred and eventually shunting procedure was performed.
Discussion: Clinician must determine whether the priapism is ischemic or non-ischemic in order to initiate appropriate management. Ischemic priapism affects people with hematological disorders while non-ischemic priapism is commonly related to penile trauma. Cavernous blood gas is one of the diagnostic methods to distinguish between the two. Blood gas in ischemic priapism will have pO2 <30mmHg, pCO2 >60mmHg and pH <7.25. Whereas blood gas with non-ischemic priapism is similar to the arterial blood gas. Ischemic priapism is a compartment syndrome requiring immediate intervention. The American Urological Association recommended a stepwise approach beginning with intracavernous aspiration, injection of an alpha-adrenergic sympathomimetic followed by surgical shunt. Systemic treatment of the underlying disorder is administered concurrently.
Conclusion: Priapism is urology emergency that requires immediate evaluation and urgent intervention as it poses risk of permanent erectile dysfunction.
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