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Diagnostic and prognostic markers in tumor stage mycosis fungoides and Sézary syndrome
The studies described in this thesis focused on identifying diagnostic and prognostic markers in tumor stage mycosis fungoides (stage IIB) and Sézary syndrome. Sézary syndrome is a type of cutaneous T-cell lymphoma, characterized by an erythroderma, lymphadenopathy and malignant T cells in skin, lymph nodes and blood. Especially in the early stages of disease, it can be difficult to differentiate Sézary syndrome from erythrodermic inflammatory dermatoses. The studies identified immunohistochemical (TOX), immunophenotypic (CD7, CD26), molecular (STAT4, TWIST1, DNM3/ PLS3) and epigenetic (CMTM2) markers that are useful as additional diagnostic markers to discriminate Sézary syndrome from erythrodermic inflammatory dermatoses. Of the molecular markers, PLS3 expression was an important prognostic factor in Sézary syndrome. In addition, it was shown that the number of tumors and time interval between tumor formation differs greatly...
Show moreThe studies described in this thesis focused on identifying diagnostic and prognostic markers in tumor stage mycosis fungoides (stage IIB) and Sézary syndrome. Sézary syndrome is a type of cutaneous T-cell lymphoma, characterized by an erythroderma, lymphadenopathy and malignant T cells in skin, lymph nodes and blood. Especially in the early stages of disease, it can be difficult to differentiate Sézary syndrome from erythrodermic inflammatory dermatoses. The studies identified immunohistochemical (TOX), immunophenotypic (CD7, CD26), molecular (STAT4, TWIST1, DNM3/ PLS3) and epigenetic (CMTM2) markers that are useful as additional diagnostic markers to discriminate Sézary syndrome from erythrodermic inflammatory dermatoses. Of the molecular markers, PLS3 expression was an important prognostic factor in Sézary syndrome. In addition, it was shown that the number of tumors and time interval between tumor formation differs greatly among patients with mycosis fungoides stage IIB and these differences correlate with survival. Patients that developed four or more tumors during the first 6 months after diagnosis of stage IIB have a poor prognosis.
Show less- All authors
- Boonk, S.E.
- Supervisor
- Vermeer, M.H.; Willemze, R.
- Co-supervisor
- Tensen, C.P.
- Committee
- Dijk, M.R. van; Sigurdsson, V.; Veelken, J.H.
- Qualification
- Doctor (dr.)
- Awarding Institution
- Medicine, Leiden University Medical Center (LUMC) , Leiden University
- Date
- 2017-11-01
- ISBN (print)
- 9789074013147
Funding
- Sponsorship
- EORTC study (in Leiden) was supported by research funding from the Dutch Cancer Society to M.H. Vermeer.