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

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Author

Nielsen, Pia Rude ; Eriksen, Jens Ole ; Wehkamp, Ulrike ; Lindahl, Lise Maria ; Gniadecki, Robert ; Fogh, Hanne ; Fabricius, Susanne ; Bzorek, Michael ; Ødum, Niels ; Gjerdrum, Lise Mette. / Clinical and Histological Characteristics of Mycosis Fungoides and Sézary Syndrome : A Retrospective, Single-centre Study of 43 Patients from Eastern Denmark. In: Acta Dermato-Venereologica. 2019 ; Vol. 99, No. 13. pp. 1231-1236.

Bibtex

@article{52851acdf08440718a15aeac8c2ef17b,
title = "Clinical and Histological Characteristics of Mycosis Fungoides and S{\'e}zary Syndrome: A Retrospective, Single-centre Study of 43 Patients from Eastern Denmark",
abstract = "Diagnosis of mycosis fungoides and S{\'e}zary syndrome can be very challenging. Clinical and histopathological data for patients with mycosis fungoides and S{\'e}zary 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 S{\'e}zary 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 S{\'e}zary syndrome in a well-characterized Danish cohort.",
author = "Nielsen, {Pia Rude} and Eriksen, {Jens Ole} and Ulrike Wehkamp and Lindahl, {Lise Maria} and Robert Gniadecki and Hanne Fogh and Susanne Fabricius and Michael Bzorek and Niels {\O}dum and Gjerdrum, {Lise Mette}",
year = "2019",
month = dec,
day = "1",
doi = "10.2340/00015555-3351",
language = "English",
volume = "99",
pages = "1231--1236",
journal = "Acta Dermato-Venereologica",
issn = "0001-5555",
publisher = "Forum for Nordic Dermato-Venereology",
number = "13",

}

RIS

TY - JOUR

T1 - Clinical and Histological Characteristics of Mycosis Fungoides and Sézary Syndrome

T2 - A Retrospective, Single-centre Study of 43 Patients from Eastern Denmark

AU - Nielsen, Pia Rude

AU - Eriksen, Jens Ole

AU - Wehkamp, Ulrike

AU - Lindahl, Lise Maria

AU - Gniadecki, Robert

AU - Fogh, Hanne

AU - Fabricius, Susanne

AU - Bzorek, Michael

AU - Ødum, Niels

AU - Gjerdrum, Lise Mette

PY - 2019/12/1

Y1 - 2019/12/1

N2 - 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.

AB - 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.

U2 - 10.2340/00015555-3351

DO - 10.2340/00015555-3351

M3 - Journal article

C2 - 31620804

VL - 99

SP - 1231

EP - 1236

JO - Acta Dermato-Venereologica

JF - Acta Dermato-Venereologica

SN - 0001-5555

IS - 13

ER -

ID: 58955669