title: Combination therapy with extracorporeal photopheresis, interferon-alpha, PUVA and topical corticosteroids in the management of Sézary syndrome.
abstract: BACKGROUND
  Extracorporeal photopheresis (ECP) is recommended for the treatment of Sézary syndrome (SS), the leukemic variant of cutaneous T-cell lymphoma (CTCL). Several combination therapies are used to increase response rates to ECP.
  
  
  METHODS
  We report our experience with the combination therapy of ECP, interferon-alpha, PUVA and topical corticosteroids in SS.
  
  
  RESULTS
  The treatment outcome in 12 SS patients was retrospectively analyzed and showed an overall response rate to this combination treatment of 42 % with 4/12 patients achieving a partial remission and 1/12 patients a stable disease. The median overall survival time was 42 months. We investigated several clinical and laboratory parameters as an indicator for a response to treatment in our patient cohort. A combined analysis of the erythroderma assessment scale, WBC, LDH, CD4/CD8 ratio and the number of Sézary cells revealed that a reduction of several parameters significantly correlated with response to treatment. The parameters which correlated best with response were number of Sézary cells, CD4/CD8 ratio and WBC.
  
  
  CONCLUSIONS
  The investigated combination therapy was effective and well-tolerated in a subgroup of SS patients but needs to be evaluated in a larger patient population.
authors.affiliation: Department of Dermatology, Venereology and Allergology, University Medical Centre Mannheim, Ruprecht-Karls-University of Heidelberg, Mannheim, Germany. nina.booken@umm.de
authors.forename: Nina
authors.initials: N
authors.lastname: Booken
authors.forename: Christel
authors.initials: C
authors.lastname: Weiss
authors.forename: Jochen
authors.initials: J
authors.lastname: Utikal
authors.forename: Moritz
authors.initials: M
authors.lastname: Felcht
authors.forename: Sergij
authors.initials: S
authors.lastname: Goerdt
authors.forename: Claus-Detlev
authors.initials: CD
authors.lastname: Klemke
mesh_terms: D000287:Administration, Topical; D000305:Adrenal Cortex Hormones / Q000008:administration & dosage*; D000368:Aged; D000369:Aged, 80 and over; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D016898:Interferon-alpha / Q000008:administration & dosage*; D008297:Male; D008875:Middle Aged; D011701:PUVA Therapy / Q000379:methods; D017893:Photopheresis / Q000379:methods*; D012751:Sezary Syndrome / Q000188:drug therapy* / Q000473:pathology*; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D007166:Immunosuppressive Agents; D016898:Interferon-alpha