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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.
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页码:1231 / 1236
页数:6
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