Regular automated red cell exchange transfusion in the management of stuttering priapism complicating sickle cell disease
Year: 2016
Type: article
Abstract: Stuttering priapism (SP) is a painful, unwanted erection lasting up to 3 h (Broderick, 2012). It affects up to 30–45% of male patients with Sickle Cell Disease (SCD) with a prevalence of 75–89% (Adeyoju et al, 2002; Nolan et al, 2005). Management measures include urination, analgesia, exercise and sympathomimetic agents, such as etilefrine hydrochloride (EH). SP usually occurs in the early morning hours with consequent loss of sleep, daytime somnolence and anxiety, which have a negative impact on patient quality of life (Karsenty et al, 2005). SP can evolve into acute major priapism (MP) (Adeyoju et al, 2002), which carries a considerable risk of erectile dysfunction (ED) (Ballas & Lyon, 2015). Automated red cell exchange transfusion (ARCET) has been used in the management of MP and is recognized by the American Society of Apharesis (ASFA) for managing acute priapism (category III) (Ballas & Lyon, 2015). Data on its use in SP is more limited. Here, we report our experience on the effect of ARCET in five patients with SCD and recurrent SP. Patient characteristics and haematological parameters are summarised in Table 1. All procedures were performed as day cases by nurses trained to use the Spectra Optia Apheresis System (TerumoBCT Europe NV, Zaventem, Belgium). The target post-transfusion HbS was less than 10%. Red cell units were provided by the UK National Blood Service as per UK national standards for patients with SCD (Milkins et al, 2013). All five subjects reported the onset of SP between the ages of 10–25 years with duration and frequency worsening with time. All subjects had been trialled on EH at varying doses but this was not always effective or tolerated (Table 2). Exercise & warm bath EH (25–125 mg) Exercise EH (50–80 mg) ↓ frequency and duration of SP Effects short lived ↓ frequency and duration of SP Effects short lived Immediate cessation Recurrence 6 weeks post-ARCET Immediate cessation Recurrence 6 weeks post-ARCET Immediate cessation Recurrence 6 weeks post-ARCET Immediate cessation Recurrence from week 4 post-ARCET Subject 1 had been on EH since 2004 with a suboptimal response. He started ARCET in June 2011 for management of frequent painful crises and noticed an immediate cessation of symptoms. He discontinued ARCET in February 2014 due to personal reasons and was commenced on hydroxycarbamide. Even though this was effective in preventing his painful crises, it resulted in recurrence of SP to pre-ARCET frequency, duration and severity. In March 2015 he suffered an ischaemic stroke (IS) and re-commenced ARCET for secondary stroke prevention (SSP). He reported immediate cessation of SP and recurrence towards the end of his 8-weekly ARCET cycle. The same pattern was observed after subsequent cycles. Subject 2 started experiencing episodes of SP in 2011 after having suffered an isolated episode of MP in 2001. He was started on EH with no effect. He sustained a second MP episode in January 2012 after which he commenced on cyproterone acetate (CA). Even though effective, it induced erectile dysfunction and gynaecomastia and was subsequently stopped. He suffered an acute IS and was started on ARCET for SSP in October 2012. This resulted in immediate cessation of SP and recurrence towards the end of the cycle. The same pattern was observed with subsequent cycles. Subject 3 started experiencing episodes of SP in January 2004 having suffered an isolated episode of MP in 1985. He was tried on EH with little benefit. He suffered an IS in May 2011 and was started on hydroxycarbamide as he was unwilling to have transfusions at the time. He re-considered and commenced ARCET for SSP in June 2015. He too, reported immediate cessation of SP after ARCET and recurrence towards the end of the cycle. With time, and as his HbS stabilised at low levels, he remained free of SP throughout. Subject 4 reported worsening of his episodes of SP in his early twenties. EH was initially effective but he soon started having SP episodes despite treatment. He was started on ARCET for recurrent leg ulceration in 2011. This resulted in cessation of his SP. Subject 5 experienced daily SP with duration of up to 2 hs, greatly affecting his sleep and quality of life. He had been started on EH in July 2011 for worsening SP but his compliance was poor due to palpitations and headaches at night. Given our experience with previous patients who noticed great improvement in SP when treated by ARCET for other indications, we recommended ARCET for management of his SP. Like the other patients, he reported immediate cessation of SP after ARCET with recurrence between weeks 4 and 5 post-ARCET. With subsequent cycles the SP episodes ceased post-ARCET and worsened in terms of frequency and severity towards the end of the cycle but this effect was less prominent. This patient had also previously reported neuropathic pain affecting both shins. He was treated with pregabalin with good clinical response but associated with significant somnolence. Interestingly, his neuropathic pain also ceased after ARCET and recurred towards the end of his ARCET cycle. This is a single-centre report on five patients with SCD and recurrent SP treated with ARCET. For four of these patients the primary indication for ARCET was not SP. All five patients were severely affected by SP and they all experienced significant improvement, demonstrating a similar pattern of response: immediately after undergoing ARCET they were completely free of any episodes of SP with recurrence as the HbS percentage increased towards the end of the ARCET cycle, suggesting a direct correlation between SP and the HbS level. With subsequent cycles, the majority remained essentially free of SP and none of these patients required any pharmacological intervention. This pattern of immediate clinical response after ARCET and recurrence of symptoms as HbS levels rise is similar to the pattern of response observed in SCD patients with pulmonary hypertension (Tsitsikas et al, 2014) but distinctly different from the gradual response observed for recurrent pain (Tsitsikas et al, 2016). The 2016 UK National Institute for Health and Care Excellence (NICE) report recommends ARCET as a cost-effective intervention in SCD (NICE 2016) while in the UK incentives for expansion of its use are already in place (https://www.england.nhs.uk/wp-content/uploads/2016/03/bi3-auto-exchng-transfsn-sickle-cell.pdf). As SP affects a large number of patients with SCD with significant impact on their quality of life, ARCET should be considered for patients with severe SP, especially if pharmacotherapy is poorly tolerated or ineffective. AE designed the project and wrote the paper, LB collected data and co-authored the paper, RJA critically reviewed the paper and DAT designed the project and critically reviewed the paper. None of the authors have any conflict of interest to declare.
Source: British Journal of Haematology
DOI: 10.1111/bjh.14393
Authors: Anietie Ekong, Lauren Berg, Roger J. Amos, Dimitris A. Tsitsikas
Language: English
Subfield: Genetics (clinical)
Field: Medicine
Domain: Health Sciences
Open Access status: bronze
OpenAlex ID: W2531887134