Stuttering priapism in a patient with sickle cell trait treated with automated red cell exchange transfusion

Abstract

AbstractSickle cell trait (SCT) is typically an asymptomatic carrier state, but sickling complications can occur under extreme conditions. Priapism is known to be associated with sickle cell disease (SCD); The link with SCT is less well established. We report the case of a 19-year-old man with SCT presenting with prolonged priapism and a refractory, stuttering course requiring multiple invasive procedures over a 5-day period with no clear alternative triggers. In light of ongoing, stuttering priapism, he underwent red blood cell (RBC) exchange transfusion with decrease of hemoglobin S from 45.8% to 11.7%. This was followed by immediate and sustained cessation of stuttering priapism, with no further episodes at 5 months. Multiple cases of priapism associated with SCT have been reported in the literature. In most cases invasive interventions were required but RBC exchange was not attempted. RBC exchange has been reported in 2 people with exertional rhabdomyolysis in the context of SCT, with improvement in 1 case. In patients with SCT and priapism, conservative measures are used to treat brief episodes, but invasive management is required for persistent or prolonged episodes. RBC exchange transfusion may be considered for treatment of refractory, stuttering priapism in individuals with SCT.