Clinical and Histological Characteristics of Mycosis Fungoides and Sézary Syndrome: A Retrospective, Single-centre Study of 43 Patients from Eastern Denmark
DOI:
https://doi.org/10.2340/00015555-3351Keywords:
mycosis fungoides, Sezary syndrome, cutaneous T-cell lymphoma, non-Hodgkin lymphomaAbstract
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.
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Copyright (c) 2019 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
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