Algorithm for initial management of priapism in chronic myeloid leukaemia

Rodgers et al (2012) provided an excellent overview on the management of priapism, but we feel it is not sufficiently specific to the management of priapism as a presenting feature in chronic myeloid leukaemia (CML). Moreover, a review of this serious medical syndrome is likely to be of special relevance to resource-constrained regions of the world where CML frequently presents in advanced phases (Gupta et al, 1987; Tazi, 2009). Some of the diagnostic and therapeutic recommendations described by Rodgers et al (2012) may be impractical as the required facilities, especially in the developing world, may not be readily available. In a patient presenting with priapism, the presence of splenomegaly should raise the possibility of CML or a related haematological disorder. A full blood count with peripheral film examination would strongly support underlying CML. The differential diagnosis will include a myeloproliferative neoplasm or acute leukaemia. These simple tests will permit the health care team to direct relevant therapy. In contrast, although penile blood gases and color duplex ultrasound are important to differentiate between ischaemic and non-ischaemic mechanisms of priapism, we disagree that these are essential investigations in CML, as priapism in CML is already known to be ischaemic in nature (Broderick et al, 2010). We agree that leukapheresis may be used as a complement to systemic chemotherapy. The American Society for Apheresis recommends leukaphereis in acute leukaemia presenting with hyperleucocytosis, as a single leucocytapheresis can reduce the white blood cell count by 30–60% (Szczepiorkowski et al, 2010). By extrapolation, apheresis is also recommended in CML presenting with priapism (Ponniah et al, 2004; Szczepiorkowski et al, 2010). However, due to logistical limitations and delays in referral, apheresis can rarely be initiated immediately. Rodgers et al (2012) advise against the use of oral sympathomimetics without citing supporting literature. Within limitations, there are studies suggesting that oral terbutaline may be of use in pharmacologically-induced priapism (Lowe & Jarow, 1993; Priyadarshi, 2004). Oral sympathomimetics, such as etilferine, phenylephrine, metaraminol and terbutaline, are superior to placebo if administered within a short timeframe (<4 h) after onset of priapism, achieving detumescence in one-third of patients (Tay et al, 2012). While we agree that the evidence for their efficacy is weak in CML, and specific measures should not be delayed while awaiting a response, we suggest that oral sympathomimetics may be attempted while other measures are being undertaken. If successful, sympathomimetics would be a simple alternative to more expensive or invasive options. In our Canadian province, of the 60 cases of CML diagnosed in the last 4 years, 2 (3·3%) men presented with priapism, one of whom was left with permanent erectile impairment. This prompted us to develop an accessible, team-based guideline for the emergency management of this rare but serious complication of CML (Fig 1). The most important components of this approach are relatively inexpensive, and should be readily applicable in most hospital settings worldwide. This letter was written by Dr L. Chisick with direction and collaboration from Drs R. Kumar and M. Seftel. Revisions and subsequent drafts were edited and revised by Drs L. Chisick, R. Kumar and M. Seftel.

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