Priapism before and after hematopoietic stem cell therapy in individuals with sickle cell disease
Abstract
Priapism, defined as a persistent, painful erection with or without arousal, is a common complication of sickle cell disease (SCD) that affects children, adolescents, and adults. The prevalence of priapism in patients with SCD has been reported to be 32.6% 1 to 38%. 2 The comorbidities associated with priapism include permanent penile deformities, penile scarring, increased rates of sexual or erectile dysfunction, decreased overall sex life satisfaction, anxiety, sadness, depression, embarrassment, and fear. 1 Idris et al 1 demonstrated that as many as 50% of individuals with SCD never seek medical attention for their priapism events, indicating that the morbidity burden is likely higher than reported in the literature. Recently, a phase 2 randomized controlled trial 3 demonstrated that hydroxyurea (HU) combined with a placebo and hydroxyurea combined with tadalafil produced a 58.3% and 66.3% reduction in the incidence rate of priapism, respectively. This trial was the first to show an effective strategy to decrease the incidence of SCD-related priapism, Hematopoietic stem cell transplantation (HSCT) and gene therapy (ovotibeglogene autotemcel and ovotibeglogene autotemcel) are currently the only established curative therapies for SCD; however, the benefit of curative therapy in preventing further priapism episodes is not well defined. We conducted a multisite case series of individuals with SCD who had experienced priapism episodes before HSCT from 3
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Evidence Hub reads this as a randomized trial, done in people. Its evidence level is 2 of 5 (High · randomized trial). Randomized trials of a reasonable size are the strongest single-study design.
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