Treatment of extensive chronic graft-versus-host disease with extracorporeal photochemotherapy

Abstract

Extracorporeal photochemotherapy (ECP; photopheresis), an immunomodulatory therapy developed for cutaneous T-cell lymphoma, has shown promise in treating chronic graft-versus-host disease (cGvHD) in uncontrolled studies. The purpose of this study was to further examine the effects of ECP on cGvHD. ECP (administered initially 3 times weekly on alternating days) was retrospectively evaluated in 14 patients with extensive cGvHD following allogeneic hematopoietic stem cell transplantation. The median time from transplantation to ECP initiation was 29 months (range, 5-96 months). The median number of concomitant baseline treatments per patient was 3 (range, 0-5). During a median ECP duration of 17 months (range, 3-44 months), 3 patients (21%) achieved a complete cutaneous response (100% improvement), 4 patients (29%) achieved a partial cutaneous response (> or =50% improvement), and 7 patients (50%) had stable skin disease. The median time to response was 6 months (range, 2-15 months), and the median response duration was 5 months (range, 1-31 months). At endpoint, responses were ongoing in 4 patients. Resolution or improvement was noted in arthralgia (5/7 patients), oral changes (3/7), elevated liver enzymes (3/5), dry eyes (2/5), joint stiffness (3/3), pulmonary disease (1/3), and thrombocytopenia (1/1). Because of a favorable response, 11 of 13 patients (85%) who received prednisone at baseline were able to taper (7/13; 54%) or discontinue (4/13; 31%) this medication, and 12 of 14 patients (86%) were able to taper (11/14; 79%) or discontinue (1/14; 7%) ECP. Five-year posttransplantation survival was 77%. Our results suggest that adjunctive ECP improves cutaneous and extracutaneous manifestations of cGvHD and has a steroid-sparing effect.

Bronchiolitis obliterans after allo-SCT: clinical criteria and treatment options

“…86 Though several studies have been performed in patients with BO after HSCT [61][62][63][64][65][87][88][89][90][91] and lung transplantation, [92][93][94] the studies are difficult to interpret because of the heterogeneity in the treatment schedules, diagnostic criteria and response assessment criteria (Table 1). A prospective randomised study of extracorporeal photopheresis treatment performed by Flowers et al 61 revealed a significant improvement of cGVHD in the skin, and indicated a steroid sparing effect on cGvHD in general.…”

Extracorporeal photopheresis in the management of graft-versus-host disease

“…Although research continues on the biology and treatment of cgvhd, no novel therapy currently in trials offers a foreseeable and significant advance over the current state of the art. Photopheresis is a therapy that emerged in the early 2000s for the management of steroid-refractory gvhd because of its steroid-sparing ability, low associated toxicity, and efficacy in some clinical settings 6,[16][17][18][19] .…”

“…The eighteen papers retained [6][7][8][9][10][11][16][17][18][19][20][21][22][23][24][25][26][27] included, for adults, one consensus report based on a systematic review 22 , one randomized controlled trial (rct) 18 , one crossover rct 19 , one prospective cohort study 9 , three retrospective cohort studies 8,16,17 , one case series with historical controls 11 , and four case series 6,21,24,27 ; and for pediatric patients, one clinical practice guideline 23 (which also contained case-series data), one nonrandomized controlled trial 10 , one prospective cohort study 26 , and four case series 7,20,23,25 (including the series from the practice guideline). Of those eighteen papers, fourteen were identified using the medline (Ovid) database, three were submitted from the files of the lead author (CB), and one was identified from the reference list in one of the papers located in the search (Figure 1).…”

“…The patient diagnoses varied, but the typical population comprised patients with gvhd in whom either steroid treatment 6,9,21 or immunosuppressive therapy 24,27 had failed. Where an ecp device was reported, the only one described was either the uvar or uvar xts system by Therakos (Raritan, NJ, U.S.A.) 6,9,[16][17][18]21,27 . The duration for which patients received ecp treatment varied greatly, from a low of 2 weeks (median not reported) in the prospective cohort study by Greinix et al 9 to a high of 528 weeks (median: 68 weeks) in the retrospective cohort study by Bisaccia et al 17 .…”

“…Where an ecp device was reported, the only one described was either the uvar or uvar xts system by Therakos (Raritan, NJ, U.S.A.) 6,9,[16][17][18]21,27 . The duration for which patients received ecp treatment varied greatly, from a low of 2 weeks (median not reported) in the prospective cohort study by Greinix et al 9 to a high of 528 weeks (median: 68 weeks) in the retrospective cohort study by Bisaccia et al 17 . The most commonly reported outcome was the response rate, followed by survival, treatment-related mortality (trm), safety, quality of life, and the effect of ecp on various measures of gvhd by affected site ( Table i).…”