Poor prognostic factors of Sezary syndrome: A retrospective single-center study from Japan

Objectives: Although the prognosis of Sezary 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), Sezary cell count, Sezary 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 (>= 19,867pg/ml), and higher Sezary cell count (>= 7480/mu l) and Sezary 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.