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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 1 , Jens Ole ERIKSEN 1 , Ulrike WEHKAMP 2 , Lise Maria LINDAHL 3 , Robert GNIADECKI 4 , Hanne FOGH 4 ,
Susanne FABRICIUS 5 , Michael BZOREK 1 , Niels ØDUM 6 and Lise Mette Rahbek GJERDRUM 1
1
Department of Pathology, Zealand University Hospital, Roskilde, Denmark, 2 Department of Dermatology, University Hospital, Schleswig-
Holstein, Kiel, Germany, 3 Department of Dermatology, Aarhus University Hospital, Aarhus, 4 Department of Dermatology, Bispebjerg and
Frederiksberg Hospital, Copenhagen, 5 Department of Dermatology, Zealand University Hospital, Roskilde, and 6 Leo Foundation Skin Immunology
Research Center, Department of Immunology and Microbiology, University of Copenhagen, Copenhagen, Denmark
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 stu­
dy 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% progres­
sed to a higher disease stage, and risk of disease pro­
gression was higher for stage IB than IA (p  = 0.01).
All cases displayed some degree of epidermotropism
and the infiltrates consisted of pleomorphic lymphocy­
tes 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-characteri­
zed Danish cohort.
Key words: mycosis fungoides; Sézary syndrome; cutaneous
T-cell lymphoma; non-Hodgkin lymphoma.
Accepted Oct 16, 2019; E-published Oct 17, 2019
Acta Derm Venereol 2019; 99: 1231–1236.
Corr: Pia Rude Nielsen, Department of Pathology, Zealand University
Hospital, Sygehusvej 9, DK-4000 Roskilde, Denmark. E-mail: piarude@
dadlnet.dk
C
utaneous T-cell lymphoma (CTCL) represents a
complex group of disorders with various clinical
manifestations, outcomes and therapeutic considerations.
Mycosis fungoides (MF) is the most common type of
CTCL. MF represents more than 50% of all primary cu-
taneous lymphomas, and the reported incidence is up to
6.4 per million people per year in US (1). In early-stage
disease, which can last for several years, MF presents as
erythematous skin patches and/or plaques, often resemb-
ling benign inflammatory conditions. For many patients,
the disease never progresses beyond this point; however,
up to one-third of patients develop ulcerating tumours or
erythroderma, and possible further dissemination of MF
to the lymphoid system, blood and internal organs (2, 3).
Sézary syndrome (SS) is a rare subtype of CTCL, which
is often considered as the leukaemic variant of MF. SS is
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 syndro-
me 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.
defined as a combination of lymphadenopathy, exfoliative/
pruritic erythroderma and circulating neoplastic T cells in
the blood (2). The clinical and histological presentation of
MF/SS can be challenging due to similarities with several
benign inflammatory diseases (4). The histopathological
findings in MF are heterogeneous and can vary between
patients and within the same patient (5, 6). In keeping with
this, the diagnostic work-up is often prolonged and several
biopsies are needed before a final diagnosis is reached (7).
A diagnostic algorithm proposed by Pimpinelli et al. (8),
and reviewed by Vandergriff et al. (9) based on clinical,
histopathological, immunophenotypical and molecular
parameters has been proposed to add diagnostic accuracy
in early-stage disease. The aim of the present retrospective
study was to characterize the clinical, epidemiological,
histological, immunophenotypical and molecular findings,
as well as therapeutic regimens, in patients with MF or
SS, diagnosed in the region of Zealand in the eastern part
of Denmark. In addition, differences in progression free-
survival (PFS) between stages IA and IB were analysed.
MATERIALS AND METHODS
Patients and clinical data
This study is a retrospective, descriptive single-centre study of
patients diagnosed with MF and SS in the region of Zealand,
Denmark. From the National Pathology Register (Patobank),
all patients who had been registered in Region Zealand from
January 1990 to December 2016, with a histological diagnosis
of MF, SS, CTCL, T-cell lymphoma, parapsoriasis en plaques,
lymphomatoid papulosis, atypical lymphocytic infiltration and
This is an open access article under the CC BY-NC license. www.medicaljournals.se/acta
Journal Compilation © 2019 Acta Dermato-Venereologica.
doi: 10.2340/00015555-3351
Acta Derm Venereol 2019; 99: 1231–1236