| Literature DB >> 34467825 |
Yumeng Zhang1, Lucia Seminario-Vidal2, Christa Varnadoe3, Yuanyuan Lu4, Ning Dong1, Christopher Salamanca5, Shannen Whiddon5, Janice Bennett5, Rebecca Hargis5, Hien Liu6, Michael Montejo6, Mohammad Hussaini7, Carly Harro8, Jane Messina7, Kaaron Benson7, Javier Pinilla-Ibarz5, Jose Conejo-Garcia9, Lubomir Sokol5.
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.Entities:
Keywords: Cutaneous T-cell lymphoma; Sézary syndrome; large granular lymphocytic proliferation; survival; therapy
Mesh:
Year: 2021 PMID: 34467825 PMCID: PMC9167451 DOI: 10.1080/10428194.2021.1971218
Source DB: PubMed Journal: Leuk Lymphoma ISSN: 1026-8022