ORIGINAL ARTICLE   Free access

Italian Journal of Dermatology and Venereology 2021 February;156(1):73-83

DOI: 10.23736/S2784-8671.19.06403-4

Copyright © 2019 EDIZIONI MINERVA MEDICA

lingua: Inglese

Sézary Syndrome: a clinico-pathological study of 9 cases

Vittorio BOLCATO ¹, Stefania BARRUSCOTTI ¹, Annalisa DE SILVESTRI ², Carlo F. TOMASINI ¹, Valeria BRAZZELLI ¹ ✉

¹ Unit of Dermatology, IRCCS Policlinico San Matteo Foundation, University of Pavia, Pavia, Italy; ² Biometry and Statistics, IRCCS Policlinico San Matteo Foundation, University of Pavia, Pavia, Italy

BACKGROUND: Sézary 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: Nine SS patients were retrospectively identified within 288 patients with cutaneous T-cell lymphomas (CTCLs) followed from 1977 to 2017 in the Unit of Dermatology, IRCCS Policlinico San Matteo Foundation, Pavia, Italy.
RESULTS: Nine SS patients were described: 5 males and 4 females, mean age at diagnosis 66.1 years (49-87 y), 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 β2µglobulin >3500 µg/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 β2µglobulin allow stratification of patients.

KEY WORDS: Sézary Syndrome; Lymphoma, t-cell, cutaneous; Survival; Prognosis