TY - JOUR
T1 - Advanced-stage mycosis fungoides and Sézary syndrome
T2 - Survival and response to treatment
AU - Alberti-Violetti, Silvia
AU - Talpur, Rakhshandra
AU - Schlichte, Megan
AU - Sui, Dawen
AU - Duvic, Madeleine
N1 - Publisher Copyright:
© 2015 Elsevier Inc. All rights reserved.
PY - 2015/6/1
Y1 - 2015/6/1
N2 - 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.
AB - 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.
KW - Cutaneous T-cell lymphoma
KW - Lactate dehydrogenase
KW - Large cell transformation
KW - Prognostic factors
KW - Stage
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U2 - 10.1016/j.clml.2015.02.027
DO - 10.1016/j.clml.2015.02.027
M3 - Article
C2 - 25817937
AN - SCOPUS:84931563808
SN - 2152-2650
VL - 15
SP - e105-e112
JO - Clinical Lymphoma, Myeloma and Leukemia
JF - Clinical Lymphoma, Myeloma and Leukemia
IS - 6
ER -