title: Advanced-stage mycosis fungoides and Sézary syndrome: survival and response to treatment.
abstract: BACKGROUND
  Mycosis fungoides (MF) is the most common variant of cutaneous T-cell lymphomas. Although MF often has an indolent course, patients can progress to, or present with, advanced stage (stage IIB-IVB) MF or with the leukemic variant, Sézary syndrome (SS).
  
  
  METHODS
  We prospectively evaluated multiple prognostic variables, including demographics, age, TNMB (blood) stage, histologic features, lactate dehydrogenase (LDH), white blood cell counts, and response to treatment, in 168 patients with advanced-stage MF and SS from 2007 to June 2014. Kaplan-Meier estimates were used to determine the median overall survival (OS) and disease-specific survival (DSS). A Cox proportional hazards regression model was used to assess the prognostic factors with univariate and multivariate analyses.
  
  
  RESULTS
  We analyzed 140 patients with MF and 28 with SS, whose median survival was 2.47 years. A total of 79 patients (47%) died of any cause. On univariate analysis, age, lymph node stage, and serum LDH level were significant for prognosis. On multivariate analysis, skin and node stage, age, large cell transformation, and LDH level were significantly associated with worse OS. Only N stage and LDH were significant for DSS. Patients who had received biologic response modifiers and histone deacetylase inhibitors first had better survival (2.5 years) than the patients initially treated with multiagent chemotherapy (9 months).
  
  
  CONCLUSIONS
  We found that only a few factors can predict OS and DSS for patients with advanced MF/SS. Also, nonchemotherapy options should be preferred for front-line therapy to improve survival, outcomes, and side effects, including immunosuppression.
authors.affiliation: Department of Dermatology, University of Texas MD Anderson Cancer Center, Houston, TX. Electronic address: silvia.viole@gmail.com.
authors.forename: Silvia
authors.initials: S
authors.lastname: Alberti-Violetti
authors.affiliation: Department of Dermatology, University of Texas MD Anderson Cancer Center, Houston, TX.
authors.forename: Rakhshandra
authors.initials: R
authors.lastname: Talpur
authors.affiliation: Baylor College of Medicine, Houston, TX.
authors.forename: Megan
authors.initials: M
authors.lastname: Schlichte
authors.affiliation: Department of Biostatistics, University of Texas MD Anderson Cancer Center, Houston, TX.
authors.forename: Dawen
authors.initials: D
authors.lastname: Sui
authors.affiliation: Department of Dermatology, University of Texas MD Anderson Cancer Center, Houston, TX.
authors.forename: Madeleine
authors.initials: M
authors.lastname: Duvic
mesh_terms: D000367:Age Factors; D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D003131:Combined Modality Therapy; D018450:Disease Progression; D005260:Female; D056572:Histone Deacetylase Inhibitors / Q000008:administration & dosage; D006801:Humans; D007372:Interferons / Q000627:therapeutic use; D007770:L-Lactate Dehydrogenase / Q000097:blood; D008198:Lymph Nodes / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009182:Mycosis Fungoides / Q000097:blood / Q000401:mortality / Q000473:pathology* / Q000628:therapy*; D009367:Neoplasm Staging; D017893:Photopheresis; D011379:Prognosis; D011446:Prospective Studies; D012307:Risk Factors; D012751:Sezary Syndrome / Q000097:blood / Q000401:mortality / Q000473:pathology* / Q000628:therapy*; D015996:Survival Rate
chemical_list: D056572:Histone Deacetylase Inhibitors; D007372:Interferons; D007770:L-Lactate Dehydrogenase
keywords: Cutaneous T-cell lymphoma; Lactate dehydrogenase; Large cell transformation; Prognostic factors; Stage