Although sickle cell disease is very common in Oman, priapism is a relatively rare complication of this disease. This study was aimed to identify the clinical and laboratory risk factors that predispose sickle cell disease patients to priapism. In a retrospective, case-control study, data on 21 male sickle cell disease patients, with priapism, were compared to 20 age and sex-matched sickle cell disease patients without priapism from the hospital medical records. Specifically, the demographical, hematological, biochemical, and clinical parameters including complications attributable to priapism were studied. Means for continuous measures and independent t-test were used to evaluate the association between the parameter studied and the occurrence of priapism. Priapism occurrence was associated with low basal hemoglobin (Hb), along with an increase in other parameters such as white blood cell count (WBC) ( P = 0.010), platelet count ( P = 0.001), reticulocyte count ( P < 0.001), mean corpuscular volume (MCV) ( P < 0.001), and mean corpuscular hemoglobin (MCH) ( P < 0.001). Biochemical laboratory parameters revealed an association with an increased total bilirubin ( P < 0.001). Patients with priapism were more likely to have acute chest syndrome, stroke, pulmonary hypertension, asplenia ( P = 0.006), gallbladder stones, and consequently cholecystectomy. Blood exchange and Penile aspiration were the most frequent medical therapies ( P = 0.010 and P < 0.001 respectively). Patients with sickle cell disease who presented with priapism were young adults with low Hb, high WBC’s, platelets, reticulocytes, MCV, MCH, bilirubin, and LDH. These findings suggest an association of priapism with increased hemolysis. Impact statement Sickle cell disease is prevalent throughout the world with a 5.8% incidence of sickle cell trait among the Omani population, yet priapism was a relatively rare complication. Penile erection and detumescence are complex physiological processes, which require a delicate neurohormonal and cardiovascular response. In this cohort, young SCD patients developed priapism with a low incidence of complications. These patients had high WBCs, platelets, bilirubin, LDH, and reticulocytes with low hemoglobin as compared to controls, suggesting an increased hemolytic process. Favorable outcome was dependent on early intervention, with almost half of these patients needing surgical intervention.

Background and Purpose: Penile erection and detumescence are complex physiologic processes, which require delicate neurohormonal and cardiovascular response. Priapism is defined as a persistent and painful erection lasting longer than four hours without sexual stimulation. Stuttering priapism is characterized by a self-limited, recurrent, and intermittent erection, frequently occurring in patients with sickle cell disease(SCD). The aim of this study was to identify the clinical and laboratory features in SCD patients with priapism in Oman. Methodology: In this retrospective case-control study, medical records of all patients with SCD who developed priapism were retrieved from the hospital information system and compared in a 1: 1 ratio with age and gender matched SCD patients who did not ever had priapism. Addition information extracted include hematological and laboratory parameters, treatment, and complications. Data obtained were analyzed using IBM SPSS version 23. The study was conducted following approval from the hospital medical research and ethics committee. Results & Discussion: Amongst the forty-one SCD patients evaluated, in the 21 patients with priapism (mean age 24.7 yrs), there was a significantly higher WBC, platelet, retic counts, LDH and serum total bilirubin as compared to controls (p<0.05, student's t test). However, there was no significant differences in the incidence of vaso-occlusive crisis, acute chest syndrome, pulmonary hypertension or cerebrovascular accident (p >0.05, Chi Square test). Patients with priapism were managed with exchange transfusion (100%), aspiration (52%) and shunting (5%). Interestingly, none of these patients showed impotence, although 10% manifested with infertility. Conclusions: Priapism in this SCD cohort is a disease of the young, and seen predominantly in the hemolytic phenotype of the disease. Patients with priapism showed high WBC's, Platelets, Bilirubin, LDH and retics with a lower hemoglobin as compared to the controls. Favorable outcome is dependent on early intervention and almost half of these patients needed surgical intervention. Disclosures No relevant conflicts of interest to declare.