Case Presentation: A 51-year-old African American man with history of methamphetamine use disorder, hypertension, and stuttering priapism presented with 2 weeks of painful priapism. Over the past 3 months, the patient had 4 other episodes of priapism lasting 1-4 days that were treated with multiple corporeal aspirations and intracavernous epinephrine injections with temporary relief. He denied taking any trazodone, sildenafil or other phosphodiesterase inhibitors, and there was no recent sexual activity. His urine toxicology screen was negative. The patient underwent a Winter shunt procedure and then an Al Ghorab shunt procedure with bilateral corporal dilation. After initial detumescence, he experienced shunt failure and had recurrence of his priapism. His hematologic workup was notable for a mild anemia with hemoglobin of ~11 and a positive sickle cell screen; his peripheral smear did not show any sickle cells. Hemoglobin electrophoresis later returned with hemoglobin S (HbS) level of 37%, consistent with sickle cell trait. On hospital day 4, the patient underwent priapism takedown and insertion of a semi-rigid penile prosthesis with resolution of his priapism. He was eventually discharged with plan for further hematology follow up as an outpatient. His beta globin sequencing later returned negative for any other deletions or duplications.
Discussion: Sickle cell disease (SCD) refers to any of the conditions in which an individual inherits the sickle hemoglobin S mutation along with another mutated beta globin allele. In contrast, sickle cell trait (SCT) occurs when one normal beta globin allele is inherited along with the mutated sickle beta globin allele, producing hemoglobin AS (HbAS). While patients with SCD often experience many complications, those with SCT are generally asymptomatic carriers. Recurrent vaso-occlusion episodes are a hallmark of almost all forms of SCD and can present in the inpatient setting as acute vaso-occlusive pain crises, acute chest syndrome, dactylitis, myocardial infarction or stroke, venous thromboembolism, and priapism. However, vaso-occlusive crises are not known to commonly occur in those with SCT. Though ischemic priapism is known to be a frequent cause of morbidity in males with SCD, the link with SCT is less clear. This case describes an individual with refractory, stuttering priapism and SCT confirmed on hemoglobin electrophoresis. Further clarification of his past medical history was negative for prior vaso-occlusive episodes other than the relatively recent priapism. There were no family members with known SCD or SCT. It was also unusual that he did not have the diagnosis of or other manifestation of his SCT until middle age. While stuttering priapism is not usually seen with SCT, there have been rare cases previously noted in the literature with various instigating etiologies including trauma, drug or phosphodiesterase inhibitor use, and sexual activity. In these cases, multiple invasive interventions were required for cessation of the priapism. Our patient also required multiple procedures and ultimately a semi-rigid penile prosthesis before resolution of his priapism; RBC exchange was considered for him as well, though was not done as his confirmatory hemoglobin electrophoresis returned after his procedures were completed.
Conclusions: Although recurrent priapism is commonly associated with SCD, providers should also consider it as a possible presentation of SCT in order to appropriately screen and treat.