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Evaluation of mortality, prognostic parameters, and treatment efficacy in mycosis fungoides.
Porkert, Stefanie; Griss, Johannes; Hudelist-Venz, Mercedes; Steiner, Irene; Valencak, Julia; Weninger, Wolfgang; Brunner, Patrick M; Jonak, Constanze.
Affiliation
  • Porkert S; Department of Dermatology, Medical University of Vienna, Viena, Austria.
  • Griss J; Department of Dermatology, Medical University of Vienna, Viena, Austria.
  • Hudelist-Venz M; Department of Radiotherapy and Radiobiology, Medical University of Vienna, Viena, Austria.
  • Steiner I; Center for Medical Statistics, Informatics, and Intelligent Systems, Section for Medical Statistics, Medical University of Vienna, Viena, Austria.
  • Valencak J; Department of Dermatology, Medical University of Vienna, Viena, Austria.
  • Weninger W; Department of Dermatology, Medical University of Vienna, Viena, Austria.
  • Brunner PM; Department of Dermatology, Icahn School of Medicineat Mount Sinai, New York, NY, USA.
  • Jonak C; Department of Dermatology, Medical University of Vienna, Viena, Austria.
J Dtsch Dermatol Ges ; 22(4): 532-550, 2024 Apr.
Article de En | MEDLINE | ID: mdl-38444271
ABSTRACT
BACKGROUND AND

OBJECTIVES:

Mycosis 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

METHODS:

Hundred-forty MF patients were assessed retrospectively. Prognosis and disease progression/survival were analyzed using univariate Cox proportional hazards regression model and Kaplan-Meier estimates.

RESULTS:

Skin 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.

CONCLUSIONS:

Our 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.
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Texte intégral: 1 Collection: 01-internacional Base de données: MEDLINE Sujet principal: Tumeurs cutanées / Mycosis fongoïde Limites: Humans Langue: En Journal: J Dtsch Dermatol Ges Sujet du journal: DERMATOLOGIA Année: 2024 Type de document: Article Pays d'affiliation: Autriche Pays de publication: Allemagne

Texte intégral: 1 Collection: 01-internacional Base de données: MEDLINE Sujet principal: Tumeurs cutanées / Mycosis fongoïde Limites: Humans Langue: En Journal: J Dtsch Dermatol Ges Sujet du journal: DERMATOLOGIA Année: 2024 Type de document: Article Pays d'affiliation: Autriche Pays de publication: Allemagne