Leukaemia-associated priapism in children (LAPC): reviewing clinical outcomes and management strategies

Abstract

Background: Priapism is a prolonged penile erection for more than 4 hours unrelated to sexual stimulation. Rarely, it is the first clinical sign of an underlying haematological malignancy. A similar presentation is noted in childhood leukaemias. Although rare, it is known to occur and, if not managed early, can have poor long-term outcomes in terms of erectile function and psychosexual growth. We present a scoping review of leukaemia-associated priapism in children (LAPC).

Methodology: We researched literature using PubMed, Google Scholar, Embase, Scopus and Cochrane databases from January 1990 to 2024. Applicable search limiters were applied, and grey literature was excluded.

Results: A total of 31 articles were finally included in the review, from which 51 cases of LAPC were isolated and studied. The average age was 11.5 years, with chronic myeloid leukaemia (CML) being the most common malignancy (68.9%), and more than 71% of cases of CML with priapism were detected in the chronic phase. Twenty cases (39.2%) were managed with corporal lavage and sympathomimetic injections at the initial onset, with the rest managed with cytoreductive measures initially. Follow-up data revealed the death of three children, whereas, of those that survived, fourteen had preserved erectile functions after a variable period of time.

Conclusion: Priapism in children warrants a thorough physical examination focusing on organomegaly and a complete hemogram. Initial management should be two-pronged with a priapism-directed corporal-lavage approach and cytoreductive measures for better long-term outcomes.

Keywords: child; healthcare; leukaemia; management; outcomes assessment; priapism.

Figure 1.. The PRISMA flow diagram detailing the search of articles involving children with leukaemia-associated priapism.

Figure 2.. Algorithm for management of LAPC.