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. 2019 Jan;33(1):108-114.
doi: 10.1111/jdv.15236. Epub 2018 Sep 25.

Survival, disease progression and prognostic factors in elderly patients with mycosis fungoides and Sézary syndrome: a retrospective analysis of 174 patients

E Lebowitz  1 , S Geller  1   2 , E Flores  2 , M Pulitzer  1   3 , S Horwitz  1   4 , A Moskowitz  1   4 , M Kheterpal  1   2   5 , P L Myskowski  1   2
Affiliations

Survival, disease progression and prognostic factors in elderly patients with mycosis fungoides and Sézary syndrome: a retrospective analysis of 174 patients

E Lebowitz et al. J Eur Acad Dermatol Venereol. 2019 Jan.

Abstract

Background: Advanced age at diagnosis is considered a poor prognostic factor in mycosis fungoides (MF) and Sézary syndrome (SS).

Objective: To evaluate the outcomes and prognostic factors in patients diagnosed at an advanced age (≥65 years) with MF/SS.

Methods: Survival, progression rates and various clinical and histopathological variables were studied in a group of 174 elderly patients diagnosed with MF/SS between 1992 and 2015 at a single referral cancer center in the United States. Kaplan-Meier estimates were used to determine survival and progression and Cox proportional hazards regression univariate and multivariate models were used to identify prognostic factors.

Results: Of 174 elderly patients, 76.4% were diagnosed with early-stage (clinical stages IA-IIA) and 23.6% with late-stage MF/SS (IIB-IV). Advanced age was associated with poor overall survival, but not with disease-specific survival (DSS) or progression-free survival (PFS). Gender, increasing clinical stage, T and B classifications, elevated lactate dehydrogenase (LDH) levels and development of large cell transformation (LCT) were significant predictors of poor survival or disease progression. Patients with early-stage MF and <10% total skin involvement (T1 classification) or patch-only disease (T1a/T2a) showed better PFS with no observed disease-specific mortality. Folliculotropic MF was associated with poor DSS in patients with early-stage disease.

Conclusions: Older age at diagnosis of MF/SS does not predict worse disease-specific outcomes. Elderly patients with early-stage disease, specifically involving less than 10% of the skin surface with patches but without plaques or folliculotropism, have an excellent prognosis. However, the development of LCT is a strong prognostic indicator of poor survival in elderly patients with MF/SS.

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Conflict of interest statement

Conflicts of interest: Dr. Horwitz has received consulting fees from or is on an advisory board for Celgene, Infinity Pharmaceuticals, Kyowa Hakka Kirin, Seattle Genetics Inc, Spectrum Pharmaceuticals, Millennium Pharmaceuticals Inc, HUYA Bioscience, and Forty Seven; and has received research funding from Celgene, Infinity Pharmaceuticals, Kyowa Hakko Kirin, Seattle Genetics Inc, Spectrum Pharmaceuticals, Millennium Pharmaceuticals Inc, and ADC Therapeutics. Dr. Moskowitz has received research support from Seattle Genetics Inc and has received honorarium from Seattle Genetics and Takeda. Dr. Myskowski has received research support from Allos Therapeutics, Ligand Pharmaceuticals (Valeant), Schering-Plough Corporation, U.S. Bioscience (Medimmune), Bristol Myers Squibb, Merck, Orthobiotech, Genmab, Millenium, Jannsen. Dr. Geller, Ms. Lebowitz, Ms. Flores, Dr. Pulitzer, and Dr. Kheterpal have no conflicts of interest to declare.

Figures

Figure 1
Figure 1
Survival outcomes of elderly patients with early- and late-stage mycosis fungoides (MF). Kaplan-Meier curves for (a) Overall survival (OS) and (b) Disease-specific survival (DSS)
Figure 2
Figure 2
Disease specific survival (DSS) of elderly patients with early-stage disease. Kaplan-Meier curves for (a) plaque disease and (b) folliculotropism.
See this image and copyright information in PMC

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