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Predictors of Outcome in the Treatment of Sézary Syndrome and Erythrodermic Mycosis Fungoides With Extracorporeal Photopheresis

Seth R. Stevens, MD; Elma D. Baron, MD; Susan Masten, RN; Kevin D. Cooper, MD

Arch Dermatol. 2002;138:1347-1350.

Background  Extracorporeal photopheresis (ECP) is an effective treatment for cutaneous T-cell lymphoma. Controversy has arisen regarding its ability to improve survival rates in Sézary syndrome (SS). We describe our experience with ECP in the treatment of SS and erythrodermic mycosis fungoides, with particular emphasis on early predictors of long-term outcome.

Observations  We included 17 patients (15 with SS and 2 with erythrodermic mycosis fungoides) who received ECP as initial treatment. Four of these patients were moribund on presentation (Eastern Cooperative Oncology Group Performance Status score, 4) and underwent only 1 to 2 cycles of ECP. The median survival was 56 months for the 11 patients with SS and an Eastern Cooperative Oncology Group Performance Status score of less than 4. If all 15 patients with SS are considered, median survival was 34 months. Response after 5 months of ECP correlated with long-term survival. A low number (<6.0 x10³/µL) of circulating CD4⁺CD7^- lymphocytes correlated with response after 5 months of ECP.

Conclusions  Extracorporeal photopheresis is a safe, effective, and well-tolerated treatment for erythrodermic mycosis fungoides and SS. Low numbers of CD4⁺CD7^- cells in the circulation and a positive response after 5 months of therapy predicted long-term survival. Moribund patients are much less likely to benefit from ECP.

From the Departments of Dermatology, University Hospitals of Cleveland Research Institute/Case Western Reserve University (Drs Stevens, Baron, and Cooper) and the Louis Stokes Veterans Affairs Medical Center (Dr Stevens), Cleveland, Ohio; and the Cutaneous Lymphoma Program, Immunodermatology Unit, University of Michigan Medical Center, Ann Arbor (Ms Masten).

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