Clinical characteristics and prognostic factors of 70 patients with Sézary syndrome: a single-institutional experience at Moffitt cancer center

Abstract

Sézary syndrome (SS) is a rare and aggressive leukemic variant of cutaneous T-cell lymphoma, with a median overall survival (OS) rate of 2–4 years. Few studies have described the clinical outcome of SS patients since 2012. We retrospectively analyzed 70 patients diagnosed with SS treated at a high-volume tertiary cancer center between 2000 and 2018. Overall survival at 1 and 5 years was 84.1% and 50.7%, respectively. Univariate analyses identified older age (>65 years) and male sex as poor prognostic factors. Five patients presented with circulating large granular lymphocytic proliferation and had a favorable prognosis. Targeted therapies were effective in treating refractory/relapsed SS patients with a durable response. Therapeutic advancements and the comprehensive treatments used in a multidisciplinary clinic improved OS rates.

Keywords:

• Cutaneous T-cell lymphoma
• Sézary syndrome
• survival
• therapy
• large granular lymphocytic proliferation

Acknowledgments

Editorial assistance was provided by the Moffitt Cancer Center’s Office of Scientific Writing by Dr. Paul Fletcher & Daley Drucker. No compensation was given beyond their regular salaries. IRB approval status: Reviewed and approved by University of South Florida Institutional Review Board (Pro00024447).

Author contributions

Y.Z., L.S.V. and L.S. conceived and designed the analysis. Y.Z. and C.V. collected the data. Y.L. and Y.Z. performed the formal analysis. Y.Z. wrote the original draft. L.S., L.S.V, J.C., J.P., K.B., J.M., N.F., M.H., C.H., M.M, H.L, T.B., Q.G., C.S, R.H., S.W., J.B. critically reviewed and edited the manuscript.

Disclosure statement

L.S. serves in Kyowa-Kirin advisory board; L.S.V. receives speakers bureau from Kyowa-Kirin. The rest of authors have no potential conflict of interest.