European dermatology forum: Updated guidelines on the use of extracorporeal photopheresis 2020 – Part 2

Abstract

BackgroundFollowing the first investigational study on the use of extracorporeal photopheresis for the treatment of cutaneous T‐cell lymphoma published in 1983, this technology has received continued use and further recognition for additional earlier as well as refractory forms. After the publication of the first guidelines for this technology in the JEADV in 2014, this technology has maintained additional promise in the treatment of other severe and refractory conditions in a multidisciplinary setting. It has confirmed recognition in well‐known documented conditions such as graft‐vs.‐host disease after allogeneic bone marrow transplantation, systemic sclerosis, solid organ transplant rejection including lung, heart and liver and to a lesser extent inflammatory bowel disease.Materials and methodsIn order to further provide recognized expert practical guidelines for the use of this technology for all indications, the European Dermatology Forum (EDF) again proceeded to address these questions in the hands of the recognized experts within and outside the field of dermatology. This was done using the recognized and approved guidelines of EDF for this task. All authors had the opportunity to review each contribution as it was added.Results and conclusionThese updated 2020 guidelines provide at present the most comprehensive available expert recommendations for the use of extracorporeal photopheresis based on the available published literature and expert consensus opinion. The guidelines were divided into two parts: PART I covers Cutaneous T‐cell lymphoma, chronic graft‐vs.‐host disease and acute graft‐vs.‐host disease, while PART II will cover scleroderma, solid organ transplantation, Crohn’s disease, use of ECP in paediatric patients, atopic dermatitis, type 1 diabetes, pemphigus, epidermolysis bullosa acquisita and erosive oral lichen planus.

Enhancement of antibody-dependent cellular cytotoxicity is associated with treatment response to extracorporeal photopheresis in Sézary syndrome

“…NK cells and their impact and importance for the mode of action of ECP have recently attracted attention, especially in GvHD and organ transplant rejections, which together with SS constitute the three major approved indications for ECP therapy in Switzerland. 9,18,19 In CTCL, reduced activity of NK cells has been observed, and increased NK cell numbers upon ECP treatment have been reported, raising the hypothesis of a relative intact immune system being a baseline predictionfactor of response to ECP. 9,20 Here we demonstrate that NK-mediated ADCC is selectively enhanced in ECP responders and might serve as a reliable biomarker to objectively monitor response to ECP in patients with SS.…”

“…9,18,19 In CTCL, reduced activity of NK cells has been observed, and increased NK cell numbers upon ECP treatment have been reported, raising the hypothesis of a relative intact immune system being a baseline predictionfactor of response to ECP. 9,20 Here we demonstrate that NK-mediated ADCC is selectively enhanced in ECP responders and might serve as a reliable biomarker to objectively monitor response to ECP in patients with SS. These findings are strengthened by observations made in patients with GvHD, where low levels of NK cell activity were reported in non-responders and normal NKcell activity before therapy has been proposed as a prognostic marker for response.…”

“…Treatment schedule followed the European dermatology forum-guidelines on the use of extracorporeal photophoresis, with one ECP cycle (on two consecutive days) every 2 weeks for the first 3 months, followed by one ECP cycle every 4 weeks. 9 The peripheral blood samples were collected during routine ECP procedure right before the initiation of ECP treatment and on average after 9 months of uninterrupted ECP treatment. In detail, 69.2% (9/13) of the baseline pre-ECP samples were collected within 2 days before ECP initiation, 30.7% (4/13) samples were collected at a median of 2.5 months (range 1-7) before therapy start.…”

“…ECP is nowadays available in more than 200 centers worldwide with cited response rates for CTCL of approximately 60% and complete responses of 14-26%. 9,10 ECP is used as a monotherapy or in combination with other immunomodulatory agents, and is well-tolerated. 10,11 Given the interpatient variability of reponse to ECP, the identification of factors associated with a positive response to ECP at baseline is of great interest.…”

Analysis of extracorporeal photopheresis within the frame of the WAA register

“…The reinfused cells will activate therapeutic mechanisms that involve immunostimulatory, immunosuppressive, and immunotolerizing effects [1]. These effects are used to treat patients that suffer from various diseases such as Sézary syndrome, cutaneous T-cell lymphoma, acute and chronic graft-versus-host disease (GVHD), atopic dermatitis and erythroderma of other origin [4][5][6][7][8]. ECP has also been investigated as a preventive measure to reduce rejection of solid organs transplants such as heart [9], lung [10], and kidney [11] transplant [1,6,[5][6][7][8].…”