High clinical response rate with multimodality immunomodulatory therapy for Sézary syndrome.
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.
Richardson
Stephen K
SK
Department of Dermatology, University of Pennsylvania, Philadelphia, USA.
Lin
Julie H
JH
Vittorio
Carmela C
CC
Kim
Ellen J
EJ
Yoon
Jessica S
JS
Junkins-Hopkins
Jacqueline
J
Rook
Alain H
AH
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
D016898:Interferon-alpha; D007371:Interferon-gamma