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

被引:12
|
作者
Nielsen, Pia Rude [1 ]
Eriksen, Jens Ole [1 ]
Wehkamp, Ulrike [2 ]
Lindahl, Lise Maria [3 ]
Gniadecki, Robert [4 ]
Fogh, Hanne [4 ]
Fabricius, Susanne [5 ]
Bzorek, Michael [1 ]
Odum, Niels [6 ]
Gjerdrum, Lise Mette Rahbek [1 ]
机构
[1] Zealand Univ Hosp, Dept Pathol, Sygehusvej 9, DK-4000 Roskilde, Denmark
[2] Univ Hosp, Dept Dermatol, Kiel, Germany
[3] Aarhus Univ Hosp, Dept Dermatol, Aarhus, Denmark
[4] Bispebjerg & Frederiksberg Hosp, Dept Dermatol, Copenhagen, Denmark
[5] Zealand Univ Hosp, Dept Dermatol, Roskilde, Denmark
[6] Univ Copenhagen, Leo Fdn Skin Immunol Res Ctr, Dept Immunol & Microbiol, Copenhagen, Denmark
关键词
mycosis fungoides; Sezary syndrome; cutaneous T-cell lymphoma; non-Hodgkin lymphoma; LYMPHOMA TASK-FORCE; INTERNATIONAL-SOCIETY; EUROPEAN-ORGANIZATION; PROGNOSTIC-FACTORS; CUTANEOUS-LYMPHOMAS; UNITED-STATES; DIAGNOSIS; CRITERIA; VALIDATION; SURVIVAL;
D O I
10.2340/00015555-3351
中图分类号
R75 [皮肤病学与性病学];
学科分类号
100206 ;
摘要
Diagnosis of mycosis fungoides and Sezary syndrome can be very challenging. Clinical and histopathological data for patients with mycosis fungoides and Sezary 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 Sezary 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 Sezary syndrome in a well-characterized Danish cohort.
引用
收藏
页码:1231 / 1236
页数:6
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