Poor prognostic factors of Sézary syndrome: A retrospective single‐center study from Japan

Abstract

Objectives: Although the prognosis of Sézary syndrome (SS) is highly unfavorable, the prognostic factors have not been fully understood. In this study, we tried to investigate the prognostic factors in Japanese SS patients for the first time. Methods:We performed a retrospective cohort study of 19 SS patients who visited our hospital between January 1, 1999, and December 31, 2019. The collected clinical findings were age, gender, performance status (PS), and TNMB staging at diagnosis. TNMB staging was determined according to the International Society for Cutaneous Lymphomas and the cutaneous task force of the European Organization of Research and Treatment of Cancer criteria. The collected hematological findings were serum levels of soluble IL-2 receptor (sIL-2R), lactate dehydrogenase (LDH), thymus and activation-regulated chemokine (TARC), and immunoglobulin E (IgE), Sézary cell count, Sézary cell ratio among white blood cells, and eosinophil count in peripheral blood at diagnosis. Results:We analyzed the correlations between overall survival and various clinical and hematological findings. In the log-rank test, PS1-3, N2-3 stage, higher serum levels of LDH (≥355 IU/L), sIL-2R (≥1729 U/ml) and TARC (≥19,867 pg/ml), and higher Sézary cell count (≥7480/μl) and Sézary cell ratio among white blood cells (≥52%) at diagnosis were associated with decreased overall survival.Conclusions: This is the first report evaluating prognostic factors in Asian SS patients.This study may contribute to selecting the treatment strategy and improving survival and quality of life of Asian SS patients.