Advanced-stage mycosis fungoides and Sézary syndrome: survival and response to treatment

Introduction: Mycosis fungoides (MF) is the most common variant of cutaneous T-cell lymphomas. Although MF often has an indolent course, patients can progress to, or present with, advanced stage (stage IIB-IVB) MF or with the leukemic variant, Sézary syndrome (SS). Patients and methods: We prospectively evaluated multiple prognostic variables, including demographics, age, TNMB (blood) stage, histologic features, lactate dehydrogenase (LDH), white blood cell counts, and response to treatment, in 168 patients with advanced-stage MF and SS from 2007 to June 2014. Kaplan-Meier estimates were used to determine the median overall survival (OS) and disease-specific survival (DSS). A Cox proportional hazards regression model was used to assess the prognostic factors with univariate and multivariate analyses. Results: We analyzed 140 patients with MF and 28 with SS, whose median survival was 2.47 years. A total of 79 patients (47%) died of any cause. On univariate analysis, age, lymph node stage, and serum LDH level were significant for prognosis. On multivariate analysis, skin and node stage, age, large cell transformation, and LDH level were significantly associated with worse OS. Only N stage and LDH were significant for DSS. Patients who had received biologic response modifiers and histone deacetylase inhibitors first had better survival (2.5 years) than the patients initially treated with multiagent chemotherapy (9 months). Conclusion: We found that only a few factors can predict OS and DSS for patients with advanced MF/SS. Also, nonchemotherapy options should be preferred for front-line therapy to improve survival, outcomes, and side effects, including immunosuppression.