Combination therapy with extracorporeal photopheresis, interferon-α, PUVA and topical corticosteroids in the management of Sézary syndrome

Authors

  • Nina Booken,

    1. Department of Dermatology, Venereology and Allergology, University Medical Centre Mannheim, Ruprecht-Karls-University of Heidelberg, Mannheim, Germany
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  • Christel Weiß,

    1. Department of Statistics, University Medical Centre Mannheim, Ruprecht-Karls-University of Heidelberg, Mannheim, Germany
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  • Jochen Utikal,

    1. Department of Dermatology, Venereology and Allergology, University Medical Centre Mannheim, Ruprecht-Karls-University of Heidelberg, Mannheim, Germany
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  • Moritz Felcht,

    1. Department of Dermatology, Venereology and Allergology, University Medical Centre Mannheim, Ruprecht-Karls-University of Heidelberg, Mannheim, Germany
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  • Sergij Goerdt,

    1. Department of Dermatology, Venereology and Allergology, University Medical Centre Mannheim, Ruprecht-Karls-University of Heidelberg, Mannheim, Germany
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  • Claus-Detlev Klemke

    1. Department of Dermatology, Venereology and Allergology, University Medical Centre Mannheim, Ruprecht-Karls-University of Heidelberg, Mannheim, Germany
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Dr. med. Nina Booken
Department of Dermatology,
Venereology and Allergology
University Medical Centre Mannheim
Ruprecht-Karls-University of Heidelberg
Theodor-Kutzer-Ufer 1-3
D-68135 Mannheim
Tel.: +49-621-383-2833
Fax: +49-621-383-3815
E-Mail: nina.booken@umm.de

Summary

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.

Patients and Methods: We report our experience with the combination therapy of ECP, interferon-α, 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.

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