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Clinical Report

Clinical and Histological Characteristics of Mycosis Fungoides and Sézary Syndrome: A Retrospective, Single-centre Study of 43 Patients from Eastern Denmark

Pia Rude Nielsen, Jens Ole Eriksen, Ulrike Wehkamp, Lise Maria Lindahl, Robert Gniadecki, Hanne Fogh, Susanne Fabricius, Michael Bzorek, Niels Ødum, Lise Mette Rahbek Gjerdrum
DOI: 10.2340/00015555-3351

Preview of fully accepted paper, still not published in any volume

Abstract

Diagnosis of mycosis fungoides and Sézary syndrome can be very challenging. Clinical and histopathological data for patients with mycosis fungoides and Sézary syndrome in Denmark are limited. A retrospective study was performed in Region Zealand, Denmark from 1990 to 2016. A total of 43 patients with mycosis fungoides or Sézary syndrome were identified during the period. At the time of diagnosis the patients’ mean age was 64.3 years and 74.5% had early-stage (≤IIA) disease. The mean time from onset of skin disease to diagnosis was 4.4 years. Surprisingly, 43% progressed to a higher disease stage, and risk of disease progression was higher for stage IB than IA (p = 0.01). All cases displayed some degree of epidermotropism and the infiltrates consisted of pleomorphic lymphocytes with a T-helper (CD4+/CD8–) phenotype. This study describes, for the first time, all aspects of clinical and histopathological findings in patients with mycosis fungoides and Sézary syndrome in a well-characterized Danish cohort.

Significance

Clinical and histopathological data on the characteristics of patients with mycosis fungoides and Sézary syndrome in Denmark are limited. This retrospective study describes the epidemiological, clinical and histopathological features of 43 patients with mycosis fungoides and Sézary syndrome in the eastern part of Denmark during 1990 to 2016. Mean age and clinical stage at the time of diagnosis are in line with similar studies, but, surprisingly, 43% of the patients progressed to a higher disease stage. The risk of disease progression was higher for stage IB than IA.

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