Mycosis fungoides and Sézary syndrome.

Details

Serval ID
serval:BIB_4725444EC41C
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Mycosis fungoides and Sézary syndrome.
Journal
Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology
Author(s)
Jonak C., Tittes J., Brunner P.M., Guenova E.
ISSN
1610-0387 (Electronic)
ISSN-L
1610-0379
Publication state
Published
Issued date
09/2021
Peer-reviewed
Oui
Volume
19
Number
9
Pages
1307-1334
Language
english
Notes
Publication types: Journal Article
Publication Status: ppublish
Abstract
Mycosis fungoides (MF) and Sézary syndrome (SS) are primary cutaneous T-cell lymphomas (CTCL) with not yet fully understood etiology and pathogenesis. Conceptually, MF and SS are classified as distinct entities arising from different T helper cell subsets. MF is the most common CTCL entity, while SS is very rare. MF presents clinically with patch, plaque and/or tumor stages, but can also evolve as erythroderma, which in turn is pathognomonic for SS. SS is characterized by a detectable tumor-cell burden (Sézary cells) in the peripheral blood consistent with advanced-stage disease and a poor prognosis. In early-stage disease of MF, which is the predominant form, the prognosis is generally favorable. However, in up to 30 % of patients, there is progression of skin lesions, which can ultimately lead to visceral involvement. The histological manifestation of MF can be subtle in early-stage disease and therefore a careful clinicopathological correlation is paramount. The treatment of MF/SS is dependent on the disease stage. Therapeutic options include both skin-directed and systemic regimens. Apart from allogeneic stem cell transplantation (alloSCT), there is as yet no curative therapy for MF/SS. Accordingly, the treatment approach is symptom oriented and aims to reduce the tumor burden and improve health-related quality of life. However, the therapeutic landscape for CTCL is constantly being expanded by the discovery of novel therapeutic targets.
Keywords
Humans, Lymphoma, T-Cell, Cutaneous, Mycosis Fungoides/diagnosis, Mycosis Fungoides/therapy, Quality of Life, Sezary Syndrome/diagnosis, Sezary Syndrome/therapy, Skin Neoplasms/diagnosis, Skin Neoplasms/therapy, Sézary syndrome, cutaneous T-cell lymphoma, dermatology, mycosis fungoides
Pubmed
Web of science
Open Access
Yes
Create date
27/09/2021 11:39
Last modification date
09/10/2021 6:38
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