Abstract
Cutaneous T-cell lymphomas (CTCLs) comprise a heterogeneous group of extranodal non-Hodgkin lymphomas involving primarily the skin and mycosis fungoides is its most frequent entity. Whereas most patients show an indolent course in early disease (clinical stages IA to IIA), some patients progress to advanced disease (stage IIB or higher), and the 5-year survival rate is unfavorable: only 47% (stage IIB) to 18% (stage IVB). Except for allogeneic stem cell transplantation, there is currently no cure for CTCL and thus treatment approaches are palliative, focusing on patients’ health-related quality of life. Our aims were to review the current understanding of the pathogenesis of CTCL, such as the shift in overall immune skewing with progressive disease and the challenges of making a timely diagnosis in early-stage disease because of the lack of reliable positive markers for routine diagnostics, and to discuss established and potential treatment modalities such as immunotherapy and novel targeted therapeutics.
Highlights
Cutaneous T-cell lymphomas (CTCLs) are primary lymphomas of the skin, and the estimated incidence is about 5.6 per million[1,2]
The term CTCL comprises a group of malignancies that develop from skin-homing T cells, which are of the CD4+ T helper cell type in more than 90% of cases[1,2]
mycosis fungoides (MF) presents clinically with erythematous patches or plaques and this stage can last for many years without clinical progression and without affecting the life expectancy of the patient[4]
Summary
Cutaneous T-cell lymphomas (CTCLs) are primary lymphomas of the skin, and the estimated incidence is about 5.6 per million[1,2]. In rare cases, MF can initially present with tumors representing the disease variant “tumour d’emblée” It is still unclear why about 30% of patients with CTCL7 progress to advanced disease; currently, prediction of prognosis is based predominantly on clinical staging by using the TNMB (tumor, node, metastasis, blood) classification for MF/SS11 and there are additional roles for gender, age, blood lactate dehydrogenase concentration, folliculotropism, large-cell transformation, and the detection of clonality[10]. De Masson et al used highthroughput sequencing of the TCR beta gene and found that an increased frequency of the malignant T-cell clone in MF skin lesions is strongly correlated with reduced overall survival in patients with early-stage disease[55]. HRQoL and its maintenance in MF/SS are of utmost importance in the face of current palliative treatment approaches in the majority of cases
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