title: Long-term outcomes of patients with advanced-stage cutaneous T-cell lymphoma and large cell transformation.
abstract: Although mycosis fungoides (MF) is typically an indolent disease, patients with advanced-stage disease (stages IIB-IVB), including Sézary syndrome (SS), often have a poor outcome. A 31-year, retrospective analysis of our cutaneous lymphoma database, of 297 patients with MF and SS, was undertaken to study long-term outcomes and identify clinical predictors of outcome in patients with advanced-stage disease (ASD, n = 92) and large cell transformation (LCT, n = 22). Two-thirds of patients with ASD presented with de novo ASD. The median overall survival (OS) for ASD was 5 years with a 10-year predicted OS of 32%. Age at initial diagnosis (P = .01), tumor stage (P = .01), and clinical stage (P = .001) were found to be significant predictors of outcome. Patients who presented with de novo ASD demonstrated better outcomes that were not statistically significant than those with a prior diagnosis of early-stage MF (P = .25). Transformation developed in 22 of the 297 MF/SS patients (7.4%), with a transformation rate of only 1.4% in patients with early-stage disease, compared with stage IIB (27%) and stage IV (56%-67%) disease. The median OS from diagnosis of LCT was 2 years. We confirm that the incidence of LCT is strongly dependent on tumor stage at diagnosis, and we demonstrate a much lower overall risk of LCT than previously reported.
authors.affiliation: Division of Haematology and Medical Oncology, Peter MacCallum Cancer Centre, Melbourne, USA.
authors.forename: Suzanne O
authors.initials: SO
authors.lastname: Arulogun
authors.forename: H Miles
authors.initials: HM
authors.lastname: Prince
authors.forename: Jonathan
authors.initials: J
authors.lastname: Ng
authors.forename: Stephen
authors.initials: S
authors.lastname: Lade
authors.forename: Gail F
authors.initials: GF
authors.lastname: Ryan
authors.forename: Odette
authors.initials: O
authors.lastname: Blewitt
authors.forename: Christopher
authors.initials: C
authors.lastname: McCormack
mesh_terms: D000368:Aged; D002471:Cell Transformation, Neoplastic; D018450:Disease Progression; D005260:Female; D006801:Humans; D008207:Lymphatic Metastasis; D016403:Lymphoma, Large B-Cell, Diffuse / Q000401:mortality / Q000628:therapy*; D016410:Lymphoma, T-Cell, Cutaneous / Q000401:mortality / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging / Q000379:methods; D012042:Registries; D012189:Retrospective Studies; D012878:Skin Neoplasms / Q000401:mortality / Q000628:therapy*; D016896:Treatment Outcome