Introduction: Sezary syndrome is usually resistant to most treatments. The aim of this work was to evaluate short and long-term results of cytapheresis in patients with Sezary syndrome.
Patients and methods: Twenty-two patients with Sezary syndrome were included in the study (13 men, 9 women, man age 58 +/- 35.4 years). The mean duration of Sezary syndrome before cytapheresis was 6.5 +/- 2 months. Extra-cutaneous involvement was observed in 16 of the patients. Each patient underwent 33 +/- 63 cytapheresis sessions (total number = 685). Complete remission in "responders" was defined as the absence of clinical signs and Sezary cells < 1,000/mm3. In "non responders" partial remission was defined as incomplete regression of clinical signs and Sezary cells > 1,000/mm3 and failure as lack of improvement or worsened clinical situation.
Results: Complete remission was obtain in 10/22 patients (45.5 p. 100), partial remission in 4/22 (18 p. 100) and failure in 8/22 (36.5 p. 100). There was no significant difference between responders and non-responders for age, duration of the Sezary syndrome before cytapheresis, the number of sessions, extracutaneous involvement, or another associated treatment. In the responders, mean survival was 90 +/- 42 months. Two patients were alive at 5 years and 1 at 10 years. In the non responders, mean survival was 8 +/- 1.4 months and median survival was 11 months.
Conclusion: There was no significant evidence suggesting a difference between responders and non-responders. Nevertheless, mean survival was longer in patients who had achieved complete remission. Cytapheresis is thus justified for the treatment of patients with Sezary syndrome.