title: Extracorporeal photopheresis for the treatment of erythrodermic cutaneous T-cell lymphoma: a single center clinical experience with long-term follow-up data and a brief overview of the literature.
abstract: Extracorporeal photopheresis (ECP) is a therapeutic procedure in which leukapheresed peripheral blood mononuclear cells are exposed to ultraviolet A in the presence of the photosensitizer 8-methoxypsoralen and then reinfused. Several guidelines recommend ECP as a treatment of choice in erythrodermic primary cutaneous T-cell lymphomas (E-CTCL). However, the level of evidence is low due to the rarity of this disease and the lack of randomized controlled trials. We performed a review of the English literature, restricting our analysis to studies including erythrodermic patients and more than 10 cases. Based on these criteria, we identified 28 studies, with a total of 407 patients. The median response rate in erythrodermic patients was 63% (range 31-86%), with a complete response rate ranging between 0 and 62% (median 20%). In our experience, we treated 51 patients with E-CTCL since 1992. A clinical response was obtained in 32 of 51 patients (63%), with a 16% complete response rate. The median time for response induction was eight months (range: 1-23). The median response duration was 22.4 months (range six months to 11 years). The treatment was generally well tolerated without systemic toxicities grade III-IV. The pretreatment parameters significantly associated with a higher likelihood to obtain a clinical response were the B-score in the peripheral blood, CD4/CD8 ratio, and amount of circulating CD3+CD8+ cells. Literature data together with our personal experience clearly support the clinical activity and tolerability of ECP in patients with E-CTCL. Prospective controlled clinical trials are strongly recommended to better document the evidence.
authors.affiliation: Department of Medical Sciences and Human Oncology, Section of Dermato-Oncology, University of Turin, Turin, Italy.
authors.forename: Pietro
authors.initials: P
authors.lastname: Quaglino
authors.forename: Robert
authors.initials: R
authors.lastname: Knobler
authors.forename: Maria T
authors.initials: MT
authors.lastname: Fierro
authors.forename: Paola
authors.initials: P
authors.lastname: Savoia
authors.forename: Elena
authors.initials: E
authors.lastname: Marra
authors.forename: Paolo
authors.initials: P
authors.lastname: Fava
authors.forename: Maria G
authors.initials: MG
authors.lastname: Bernengo
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D017252:CD3 Complex / Q000032:analysis; D016516:CD4-CD8 Ratio; D016827:CD8 Antigens / Q000032:analysis; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D016410:Lymphoma, T-Cell, Cutaneous / Q000097:blood / Q000188:drug therapy* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009182:Mycosis Fungoides / Q000097:blood / Q000188:drug therapy; D017893:Photopheresis / Q000009:adverse effects / Q000379:methods*; D017410:Practice Guidelines as Topic; D012074:Remission Induction; D012189:Retrospective Studies; D012751:Sezary Syndrome / Q000097:blood / Q000188:drug therapy; D012878:Skin Neoplasms / Q000097:blood / Q000188:drug therapy* / Q000473:pathology; D013601:T-Lymphocytes / Q000737:chemistry; D013997:Time Factors
chemical_list: D017252:CD3 Complex; D016827:CD8 Antigens