UKPS Publications

Background: Extra-corporeal photopheresis (ECP) requires anticoagulation to pre- vent circuit clotting. Unfractionated heparin (UFH) is currently the only anticoagulant licensed for the ECP system in use in the United Kingdom (UK). Acid citrate dextrose-A (ACD-A) is the preferred anticoagulant for most other apheresis proce- dures. Anecdotal evidence suggested variability in ECP practice across the UK with some providers using off-label ACD-A.

Aims: We developed a survey together with the UK Photopheresis Society to estab- lish current practice.

Materials & Methods: This was distributed to all 17 ECP providers covering 34 UK sites.

Results: Significant variability in practice was demonstrated with only 36% of responding providers (5/14) using UFH exclusively and 29% (4/14) using ACD-A as standard.

Conclusion: This survey highlights the need for a UK consensus.

Overall, this audit confirms the established favorable safety profile and efficacy of ECP in cGvHD in children and adults. The rate of immunosuppression withdrawal was similarly consistent. Whilst response data are best accrued prospectively, the benefit of completing a course of ECP was evident with all patients achieving at least a partial response. In comparison, patients with progressive disease received fewer cycles of treatment, and experienced a high mortality rate, which appeared predominantly related to infection. These data highlight the importance of prompt consideration of ECP therapy in second line treatment of cGvHD, which may significantly reduce corticosteroid and immunosuppressant therapy, and reduce the infection risk.

This large audit of UK practice supports further prospective studies and clinical trials to define the best use of ECP in controlling cGvHD in the first line of treatment; for example, alongside standard of care. In addition, scientific studies of biomarkers of GvHD should be associated with prospective clinical data acquisition in both routine service and clinical trial delivery of ECP.

Extracorporeal photopheresis (ECP) is a second-line therapy in acute and chronic GVHD and solid organ transplant rejection. We report ECP use in 98 pediatric patients in seven UK centers from 2010 to 2017, the majority treated for aGVHD (73.5%). ECP was safe and well tolerated including in low body weight patients. Most patients were on multiple immunosuppressive therapies prior to ECP; 45.9% were able to reduce or stop immunosuppression with treatment. Complete or partial response was reported in almost 60%. This study supports the need to include ECP treatment data to national transplant databases to provide accurate information regarding service provision, patient outcomes, and safety.

Extracorporeal photopheresis (ECP) has been used for over 35 years in the treatment of erythrodermic cutaneous T-cell lymphoma (CTCL) and over 20 years for chronic and acute graft-versus-host disease (GvHD) and solid organ transplant rejection. ECP for CTCL and GvHD is available at specialised centres across the UK. The lack of prospective randomised trials in ECP led to the development of UK Consensus Statements for patient selection, treatment schedules, monitoring protocols and patient assessment criteria for ECP. The recent literature has been reviewed and considered when writing this update. Most notably, the national transition from the UVAR XTS^® machine to the new CELLEX machine for ECP with dual access and a shorter treatment time has led to relevant changes in these schedules. This consensus statement updates the previous statement from 2007 on the treatment of CTCL and GvHD with ECP using evidence based medicine and best medical practise and includes guidelines for both children and adults.