Abstract
Cutaneous T cell lymphomas (CTCLs) are a heterogeneous group of extranodal non-Hodgkin’s lymphomas that are characterized by a cutaneous infiltration of malignant monoclonal T lymphocytes. They typically afflict adults with a median age of 55 to 60 years, and the annual incidence is about 0.5 per 100,000. Mycosis fungoides, Sézary syndrome, and primary cutaneous peripheral T cell lymphomas not otherwise specified are the most important subtypes of CTCL. CTCL is a complicated concept in terms of etiopathogenesis, diagnosis, therapy, and prognosis. Herein, we summarize advances which have been achieved in these fields.
Highlights
In 1806, Alibert initially described mycosis fungoides (MF) as the infiltration of skin by lymphocytes
In 1974, Edelson used the term “cutaneous T cell lymphomas” (CTCLs) for MF and its leukemic variant, Sézary syndrome (SS), which are the major types of CTCL1
The Cutaneous T cell lymphomas (CTCLs), which are characterized by infiltration of malignant monoclonal T lymphocytes in the skin, are considered a heterogeneous group of extranodal non-Hodgkin’s lymphomas[1,2,3]
Summary
In 1806, Alibert initially described mycosis fungoides (MF) as the infiltration of skin by lymphocytes. Studies show that the overexpression of TOX and its protein product is associated with thicker lesions of MF, disease progression, and poor prognosis Dysregulation of this gene is reported in lesions and peripheral blood mononuclear cells of cases with SS22. HDACIs, classified as anti-neoplastic agents, are novel therapeutic options for treating CTCLs1,9,62 Their mechanisms of action are via transcription-dependent and transcription-independent ways[11], including (a) promoting the expression of genes that regulate cell differentiation and apoptosis, (b) inducing changes to the structural integrity of chromatin[1], (c) regulating miR-22 expression[25], and (d) increasing the production of reactive oxygen species and decreasing mitochondrial membrane[63]. The efficacy of topical resiquimod, an imidazoquinoline with TLR7- and TLR8-stimulating activity, was shown in treating early stage CTCLs. Its effectiveness in inducing regression of untreated lesions was reported, probably mediated by enhancing systemic anti-tumor immunity. In PCTCL-NOSs, the 5-year survival rate is less than 20%8
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