Clinical characteristics and prognostic factors of 70 patients with Sézary syndrome: a single-institutional experience at Moffitt cancer center. Issue 1 (2nd January 2022)

• Record Type: Journal Article
• Authors: Zhang, Yumeng; Seminario-Vidal, Lucia; Varnadoe, Christa; Lu, Yuanyuan; Dong, Ning; Salamanca, Christopher; Whiddon, Shannen; Bennett, Janice; Hargis, Rebecca; Liu, Hien; Montejo, Michael; Hussaini, Mohammad; Harro, Carly; Messina, Jane; Benson, Kaaron; Pinilla-Ibarz, Javier; Conejo-Garcia, Jose; Sokol, Lubomir
• Is Part Of: Leukemia & lymphoma. Volume 63:Issue 1(2022)
• Page Start: 109
• Page End: 116
• Publication Date: 2022-01-02
• Subjects: Cutaneous T-cell lymphoma -- Sézary syndrome -- survival -- therapy -- large granular lymphocytic proliferation; Leukemia -- Periodicals; Lymphomas -- Periodicals; 616.99419
• DOI: 10.1080/10428194.2021.1971218
• Languages: English
• ISSNs: 1042-8194

Abstract

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.