title: High clinical response rate with multimodality immunomodulatory therapy for Sézary syndrome.
abstract: OBJECTIVE
  The goal of this study was to evaluate the clinical response rate of patients with Sézary syndrome (SS) to multimodality immunomodulatory therapy consisting of extracorporeal photopheresis in combination with >/= 2 systemic biologic response modifiers (interferon-, interferon-, retinoids, and/or sargramostim) and psoralen plus UV-A.
  
  
  METHODS
  Twenty-eight patients who met established criteria for SS were treated with multimodality immunomodulatory therapy at the Hospital of the University of Pennsylvania between January 2000 and December 2002. All patients received > 6 cycles of extracorporeal photopheresis. Patients were categorized into groups based on their response to therapy.
  
  
  RESULTS
  An overall clinical response of 89% was achieved with multimodality immunomodulatory therapy. Twenty-nine percent of patients exhibited a complete response, characterized by no evidence of cutaneous disease and a Sézary count 5%. Sixty-one percent exhibited a partial response. Eleven percent were nonresponders.
  
  
  CONCLUSIONS
  Based on our experience, multimodality immunomodulatory therapy is an exceptionally effective treatment for SS. The durability of response and impact on overall survival remains to be determined; however, this approach offers an appealing alternative to treatments associated with higher morbidity rates.
authors.affiliation: Department of Dermatology, University of Pennsylvania, Philadelphia, USA.
authors.forename: Stephen K
authors.initials: SK
authors.lastname: Richardson
authors.forename: Julie H
authors.initials: JH
authors.lastname: Lin
authors.forename: Carmela C
authors.initials: CC
authors.lastname: Vittorio
authors.forename: Ellen J
authors.initials: EJ
authors.lastname: Kim
authors.forename: Jessica S
authors.initials: JS
authors.lastname: Yoon
authors.forename: Jacqueline
authors.initials: J
authors.lastname: Junkins-Hopkins
authors.forename: Alain H
authors.initials: AH
authors.lastname: Rook
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D003131:Combined Modality Therapy / Q000379:methods; D003713:Dendritic Cells / Q000378:metabolism; D005260:Female; D006801:Humans; D007167:Immunotherapy / Q000379:methods*; D016898:Interferon-alpha / Q000378:metabolism; D007371:Interferon-gamma / Q000378:metabolism; D008214:Lymphocytes / Q000378:metabolism; D016410:Lymphoma, T-Cell, Cutaneous / Q000276:immunology / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D012751:Sezary Syndrome / Q000628:therapy*; D012878:Skin Neoplasms / Q000276:immunology / Q000628:therapy*; D016896:Treatment Outcome
chemical_list: D016898:Interferon-alpha; D007371:Interferon-gamma