Extracorporeal Photopheresis: An Efficacious and Well-Tolerated Treatment for Cutaneous and Oral Mucosal Chronic Graft-versus-Host Disease

Abstract

Background: Extracorporeal photopheresis (ECP) is a second-line therapy for steroid-refractory chronic graft-versus-host disease (cGVHD). Objective: We describe the long-term efficacy and tolerability of ECP according to the cutaneous phenotype of cGVHD and report on the reduced need for immunosuppressant drugs in this setting. Patients and Methods: Fourteen patients (8 females) with cutaneous and/or mucosal cGVHD, treated with ECP between October 2010 and May 2016 within a single center, were included. Final analyses included patients who had received ECP for at least 12 months. We prospectively evaluated the efficacy of ECP using lesion-specific clinical scores and by recording changed doses of systemic immunosuppressants. Results: Of the 14 patients, sclerotic skin lesions were present in 10 (71%). The mRODNAN score decreased in all patients from month 9 onwards, with 40 and 77% reductions at 12 and 36 months, respectively. Six patients (43%) presented with cutaneous lichenoid lesions: this score was reduced in all patients by month 3, reaching a 93% reduction by month 12. Five patients (36%) experienced oral mucosal lichenoid lesions: these scores were decreased by 55% at month 12 and by 100% by month 33. The use of systemic immunosuppressants was reduced in all patients; 4 patients could stop all immunosuppressant drugs after 2 years. ECP was stopped in 3 patients after a complete response. No major ECP-associated adverse effects were observed. Discussion and Conclusion: ECP was an effective long-term therapy for oral and cutaneous cGVHD: consequently, dose levels of therapeutic immunosuppression could be reduced.

Efficacy and safety of one‐day offline extracorporeal photopheresis schedule processing one total blood volume for treating patients with graft‐versus‐host disease

“…We found previous studies where authors treated patients with just one ECP procedure (Table ) . Authors using inline ECP procedures collected MNCs after processing 1500 mL of whole blood . However, Garban and colleagues collected MNCs with an offline system after processing “three whole blood masses” and Iniesta and coworkers did not mention the quantity of whole blood processed in their study.…”

“…[24][25][26][27][28][29][30][31][32] Authors using inline ECP procedures collected MNCs after processing 1500 mL of whole blood. [25][26][27][28][30][31][32] However, Garban and colleagues 24 collected MNCs with an offline system after processing "three whole blood masses" and Iniesta and coworkers 29 did not mention the quantity of whole blood processed in their study. After all the previous studies were reviewed, it is obvious that there is no consensus regarding the most appropriate ECP schedule for treating patients with acute or chronic GVHD.…”

“…In the chronic GVHD schedule, we decided to treat our patients once per week, processing one TBV (median, 4720 mL) for 4 weeks, and then tapered to once every 2 weeks. We found previous studies where authors treated patients with just one ECP procedure (Table ) . Authors using inline ECP procedures collected MNCs after processing 1500 mL of whole blood .…”

Chronic Ocular Graft Versus Host Disease: An Update and Review

“…56 Another second-line therapy that is used in conjunction with immunosuppressives is extracorporeal photopheresis (ECP), especially in the cutaneous and mucosal manifestations, 59 ECP may also be useful in as a first-line therapy in patients that have contraindications to immunosuppressives such as cerebral toxoplasmosis. 60 While the mode of action is unclear, the combination of leukapheresis and photodynamic therapy has been reported to help to moderate the effects of GVHD. A multicenter trial, with results reported in 2018, showed that ECP had a 62% provider response rate and a 44% NIH criteria response rate.…”