Sézary Syndrome: a clinico-pathological study of 9 cases and review of the literature

BACKGROUND: Sezary Syndrome (SS) is a rare and aggressive variant of cutaneous T-cell lymphoma characterized by erythroderma, generalized lymphadenopathy and atypical lymphocytes in peripheral blood. The aim of the study is to describe our experience with SS patients.METHODS: 9 SS patients were retrospectively identified within 288 patients with cutaneous Tcell lymphomas (CTCLs) followed from 1977 to 2017 in the Unit of Dermatology, IRCCS Policlinico San Matteo Foundation, Pavia.RESULTS: 9 SS patients were described: 5 males and 4 females, mean age at diagnosis 66.1 years (49-87 ys), overall survival (OS) after SS diagnosis was 2.6 years (31.5 ms). All the patients showed erythroderma, pruritus and lymphadenopathy. Palmo-plantar hyperkeratosis, nail lesions, alopecia and ectropion were also present. One patient was excluded for significative differences in management. Three lines treatment -extracorporeal photopheresis plus immunomodulator/s plus photo-photochemotherapy- was the most used first-line option for induction of remission, reached in 4 patients out of 8: 3 with Complete Remission (CR), 1 with Partial Remission (PR). Prognostic variables were investigated by univariate analysis: hypereosinophilia, highly elevated beta2muglobulin >3500 mug/l, male sex and highly elevated LDH >450 U/l resulted with statistical power.CONCLUSIONS: The improved comprehension of SS pathogenesis is progressively increasing the -still poor- survival: 38.5 months (3.2 years) considering only the 6 patients followed in the last five years, versus overall 31.5 months (2.6 years). The correct identification of SS patients remains determinant for the proper overall management. Among unfavorable prognostic markers, levels of beta2muglobulin allow stratification of patients.