Case Report of Priapism in a Patient with Thalassemia Minor.

Background - Priapism is a urologic emergency caused by hematological problems in about 35% of cases. Its occurrence in sickle cell anemia, leukemia /other malignancies and primary thrombocythemias is well established. A very few case reports of priapism occurring in Thalassemia Intermedia especially after splenectomy have been documented in literature. Veno-occlusive priapism (low flow priapism) beyond 4hours is a compartment syndrome requiring emergent medical intervention. The most important late complication of priapism is fibrosis and impotence and their incidence is related to the duration and aggressiveness of treatment of priapism. We report a case of priapism in a patient of Thalassemia minor.

Case report- A 42 year Asian male was referred from urology service for a hematologic work-up for recurrent episodes of priapism. He was admitted with a 24 hours-long episode of priapism. The history of priapism dates back to 1998, a few months after marriage, when he was admitted for the first episode of priapism and had a decompressive procedure. Since then, for 6 years, he had intermittent, 15 minutes to 1 hour episodes of penile erection. These episodes usually occur after midnight and were unrelated to sexual activity. He did not have any other medical problems and was not on any medications. He was not on any pro-erectile medications. He denied any history of masturbation. He denied any history of alcohol or illicit drug use. He was unaware of any family history of anemia. On physical examination, he did not have pallor, icterus, lymphadenopathy, skin ulcers or hepatosplenomegaly. The penis was circumcised without any trauma or necrosis. His hemoglobin was 10.5 g/dl, hematocrit 34%, MCV 65fl, RDW14.5 %, reticulocyte count 1.3%, platelets 274k/μl, and WBC 6.9k/μl. Peripheral smear showed poikilocytosis, hypochromia and microcytosis without any sickle cells or nucleated RBCs. Hemoglobin electrophoresis showed Hb A of 94%, Hb A₂ of 5.6% and Hb F of 0.4%. His clinical picture, blood count, peripheral smear and electrophoresis were consistent with Thalassemia minor. He was managed by removal of inspissated blood and injection of phenylephrine into the corpora cavernosa. This was repeated a few times and the priapism was relieved and patient discharged.

Conclusion- This is the first reported case of priapism in a patient with Thalassemia minor.