Extracorporeal photopheresis for the treatment of erythrodermic cutaneous T-cell lymphoma: a single center clinical experience with long-term follow-up data and a brief overview of the literature

Authors

  • Pietro Quaglino MD,

    Corresponding author
    1. Department of Medical Sciences and Human Oncology, Section of Dermato-Oncology, University of Turin, Turin, Italy
    • Correspondence

      Pietro Quaglino, md

      Department of Biomedical Sciences and Human Oncology

      Section of Dermatology

      1st Dermatologic Clinic

      University of Turin

      Via Cherasco 23

      10126 Torino

      Italy

      E-mail: pietro.quaglino@unito.it

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    • These authors equally contributed to the paper.
  • Robert Knobler MD,

    1. Medical University of Vienna, Vienna, Austria
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    • These authors equally contributed to the paper.
  • Maria T. Fierro MD,

    1. Department of Medical Sciences and Human Oncology, Section of Dermato-Oncology, University of Turin, Turin, Italy
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  • Paola Savoia MD,

    1. Department of Medical Sciences and Human Oncology, Section of Dermato-Oncology, University of Turin, Turin, Italy
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  • Elena Marra MD,

    1. Department of Medical Sciences and Human Oncology, Section of Dermato-Oncology, University of Turin, Turin, Italy
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  • Paolo Fava MD,

    1. Department of Medical Sciences and Human Oncology, Section of Dermato-Oncology, University of Turin, Turin, Italy
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  • Maria G. Bernengo MD

    1. Department of Medical Sciences and Human Oncology, Section of Dermato-Oncology, University of Turin, Turin, Italy
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  • Conflicts of interest: None.

Abstract

Extracorporeal photopheresis (ECP) is a therapeutic procedure in which leukapheresed peripheral blood mononuclear cells are exposed to ultraviolet A in the presence of the photosensitizer 8-methoxypsoralen and then reinfused. Several guidelines recommend ECP as a treatment of choice in erythrodermic primary cutaneous T-cell lymphomas (E-CTCL). However, the level of evidence is low due to the rarity of this disease and the lack of randomized controlled trials. We performed a review of the English literature, restricting our analysis to studies including erythrodermic patients and more than 10 cases. Based on these criteria, we identified 28 studies, with a total of 407 patients. The median response rate in erythrodermic patients was 63% (range 31–86%), with a complete response rate ranging between 0 and 62% (median 20%). In our experience, we treated 51 patients with E-CTCL since 1992. A clinical response was obtained in 32 of 51 patients (63%), with a 16% complete response rate. The median time for response induction was eight months (range: 1–23). The median response duration was 22.4 months (range six months to 11 years). The treatment was generally well tolerated without systemic toxicities grade III–IV. The pretreatment parameters significantly associated with a higher likelihood to obtain a clinical response were the B-score in the peripheral blood, CD4/CD8 ratio, and amount of circulating CD3+CD8+ cells. Literature data together with our personal experience clearly support the clinical activity and tolerability of ECP in patients with E-CTCL. Prospective controlled clinical trials are strongly recommended to better document the evidence.

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