Priapism in hematological and coagulative disorders: an update

Abstract

Priapism is a true urological emergency that is typified by a persistent and painful erection. High-risk groups include patients with hematological or coagulative disorders; for example, those with sickle cell disease, leukemia or glucose-6-phosphate dehydrogenase deficiency. The diagnosis for priapism must be made urgently using patient history, physical examination and blood gas findings on corporal aspiration. Emergency treatment is needed to avoid erectile dysfunction. However, in high-risk groups, prophylaxis must be encouraged. A number of prophylactic measures are emerging based on progress in the understanding of the pathophysiology of priapism in these particular patients. In this Review, priapism as it relates to hematological disorders is discussed, focusing on treatment and prophylaxis.

Molecular Pathophysiology of Priapism: Emerging Targets

“…Intracavernosal sympathomimetics are currently employed in the treatment of ischemic priapism episodes, particularly in instances of failure of initial clinical aspiration and saline irrigation attempts, and also acutely as needed by patient self-injection for termination of the relatively shorter RIP episodes [7, 8]. On local injection, these agents function to cause alpha-adrenergic-mediated vasoconstriction through contractile stimulation of cavernous smooth muscle, resulting in detumescence [83].…”

“…Many available agents often have cardiac inotropic and chronotropic side-effects via beta-adrenergic stimulation. Accordingly, phenylephrine is typically the agent of choice due to its minimal beta-adrenergic stimulatory effects [7, 83]. …”

“…This population is at an elevated risk of recurrent ischemic priapism (RIP), also termed stuttering priapism, a variant of the common and often painful ischemic (low flow or veno-occlusive) priapism [1]. RIP, as its name implies, involves repeated ischemic episodes, which are typically transient and self-limiting, occurring during sleep and lasting less than 3 hours in duration [1, 7]. Although transient, this variant may be a harbinger of longer, major episodes as nearly 30% of RIP cases have been reported to progress to a major episode of ischemic priapism [5].…”

How I treat priapism

“…1,2 The term is derived from Priapus, a Greek god of fertility renowned for his large phallus. 3,4 Incidence rates of 1.5 per 100 000 person-years have been estimated among the general population. 5 The ischemic sub-type is common in sickle cell disease (SCD) with prevalence rates of up to 40%.…”

“…2 The natural history of RIP is heterogeneous; most episodes are transient and self-remitting, often occurring during sleep and lasting for ,3 hours. 3 However, RIP is considered a harbinger of major ischemic priapism. In fact, nearly 30% progress to major episodes 7,11 and .70% of SCD patients with a history of severe priapism have a history of RIP.…”