Evaluation of mortality, prognostic parameters, and treatment efficacy in mycosis fungoides

Author:

Porkert Stefanie1,Griss Johannes1,Hudelist‐Venz Mercedes2,Steiner Irene3,Valencak Julia1,Weninger Wolfgang1,Brunner Patrick M.4,Jonak Constanze1

Affiliation:

1. Department of Dermatology Medical University of Vienna Viena Austria

2. Department of Radiotherapy and Radiobiology Medical University of Vienna Viena Austria

3. Center for Medical Statistics Informatics and Intelligent Systems Section for Medical Statistics Medical University of Vienna Viena Austria

4. Department of Dermatology Icahn School of Medicineat Mount Sinai New York, NY USA

Abstract

SummaryBackground and ObjectivesMycosis fungoides (MF), the most common primary cutaneous T‐cell lymphoma, is characterized by a variable clinical course, presenting either as indolent disease or showing fatal progression due to extracutaneous involvement. Importantly, the lack of prognostic models and predominantly palliative therapy settings hamper patient care. Here, we aimed to define survival rates, disease prediction accuracy, and treatment impact in MF.Patients and methodsHundred‐forty MF patients were assessed retrospectively. Prognosis and disease progression/survival were analyzed using univariate Cox proportional hazards regression model and Kaplan‐Meier estimates.ResultsSkin tumors were linked to shorter progression‐free, overall survival and a 3.48 increased risk for disease progression when compared to erythroderma. The Cutaneous Lymphoma International Prognostic Index identified patients at risk in early‐stage disease only. Moreover, expression of Ki‐67 >20%, CD30 >10%, CD20+, and CD7 were associated with a significantly worse outcome independent of disease stage. Only single‐agent interferon‐α and phototherapy combined with interferon‐α or retinoids/bexarotene achieved long‐term disease control in MF.ConclusionsOur data support predictive validity of prognostic factors and models in MF and identified further potential parameters associated with poor survival. Prospective studies on prognostic indices across disease stages and treatment modalities are needed to predict and improve survival.

Publisher

Wiley

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