Folliculotropic Mycosis Fungoides with Skewed T-cell Receptor CDR3 motif: Suggestive of Lipid Antigen-selection?

Folliculotropic Mycosis Fungoides with Skewed T-cell Receptor CDR3 motif: Suggestive of Lipid Antigen-selection? Acta Derm Venereol. 2017 Jun 09;: Authors: Mantaka P, Malecka A, Trøen G, Helsing P, Gjersvik P, Beiske K, Delabie J Abstract Folliculotropic mycosis fungoides (FMF), a variant of mycosis fungoides (MF) with distinct clinical features, is characterized by infiltration of malignant T cells in hair follicles. This raises the hypothesis that antigens in the hair follicle may contribute to the pathogenesis of FMF. T-cell receptor β gene (TRB) sequences as well as dendritic cell subsets in patients with FMF (n = 21) and control patients with MF (n = 20) were studied to explore this hypothesis. A recurrent usage of the TRB junctional genes TRBJ2-1 and TRBJ2-7 was found in patients with FMF compared with those with MF. These genes contribute to an amino acid motif in the complementarity-determining region 3 (CDR3) of the T-cell receptor. This motif was previously found in T cells stimulated by lipids bound to CD1 on antigen-presenting cells. Additional immunohistochemical analysis revealed abundant CD1c- and CD1a- expressing dendritic cells in FMF. The combined findings support a role for lipid-antigen stimulation in FMF. PMID: 28597017 [PubMed - as supplied by publisher]
Source: Acta Dermato-Venereologica - Category: Dermatology Authors: Tags: Acta Derm Venereol Source Type: research

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In conclusion, cdTSE has a limited role in SS. TTNT is reduced in heavily pre-treated MF patients, suggesting greater benefit when utilized earlier in treatment sequencing.
Source: Cancers - Category: Cancer & Oncology Authors: Tags: Article Source Type: research
AbstractPurposeCutaneous T cell lymphomas (CTCL) are rare and histologically diverse lymphoproliferative neoplasms, with mycosis fungoides (MF) representing the most common disease subset. Given the emerging role of myeloid-derived suppressor cells (MDSC) as a clinically applicable biomarker in solid tumors, we sought to investigate the presence of tumor-infiltrating and circulating MDSC in early- and advanced-stage MF patients and evaluate their prognostic significance in patient overall survival.MethodsTumor-infiltrating MDSC were assessed immunohistochemically with Arginase-1 in 31 MF and 14 non-MF skin punch biopsies. ...
Source: Clinical and Translational Oncology - Category: Cancer & Oncology Source Type: research
CONCLUSIONS: The clinical roles of PD-1/PD-L1 expression vary between subtypes of lymphoma. Future studies should delineate the prognostic and predictive roles of PD-1 and PD-L1 expression. PMID: 31704289 [PubMed - as supplied by publisher]
Source: International Immunopharmacology - Category: Allergy & Immunology Authors: Tags: Int Immunopharmacol Source Type: research
The PI3K/AKT/mTOR pathway is hyperactivated in many tumors, as well as in cutaneous T-cell lymphoma (CTCL) which includes mycosis fungoides (MF) and the aggressive variant known as Sezary syndrome (SS). TORC1 signaling is activated in SS cells by cytokines and chemokines, which are overexpressed in SS tissues. Furthermore, the recurrent copy number variation (CNV) of genes belonging to this cascade, such as PTEN, LKB1, and P70S6K, contributes to the hyperactivation of the pathway. The aim of this study was to investigate the therapeutic potential of mTOR inhibitors in CTCL.
Source: Journal of Investigative Dermatology - Category: Dermatology Authors: Tags: Original Article Source Type: research
AbstractCutaneous T cell lymphoma (CTCL) is a rare but potentially devastating primary cutaneous lymphoma. CTCL is characterized by localization of neoplastic T lymphocytes to the skin, with mycosis fungoides (MF) and its leukemic form, S ézary syndrome (SS) being the most common variants. Thymocyte selection-associated high-mobility group box (TOX) gene has been found to be highly expressed in MF and SS. It is reported that higher expression levels of TOX in patients will increase risks of disease progression and poor prognosis. Ho wever, the molecular events leading to these abnormalities have not been well unders...
Source: Archives of Dermatological Research - Category: Dermatology Source Type: research
Abstract Most patients with mycosis fungoides are diagnosed with early-stage disease. However, prevalence of early-stage disease is unknown, and evidence of its burden is scarce. The aim of this study is to estimate the prevalence of early-stage mycosis fungoides, how long patients live with early-stage disease and to characterise these patients. Data were obtained from 4 key publications and from US cancer registries (Surveillance, Epidemiology and End Results Program; SEER). The derived incidence of early-stage mycosis fungoides was 0.26/100,000 (UK), 0.29/100,000 (US) and 0.38/100,000 (US-SEER) and the prevalen...
Source: Acta Dermato-Venereologica - Category: Dermatology Authors: Tags: Acta Derm Venereol Source Type: research
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Source: The Annals of Pharmacotherapy - Category: Drugs & Pharmacology Authors: Tags: Ann Pharmacother Source Type: research
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Source: Clinical Cancer Research - Category: Cancer & Oncology Authors: Tags: Clin Cancer Res Source Type: research
The objective of this study was to test the hypothesis that worse prognosis in blacks with MF is mediated by higher disease stage at diagnosis and by earlier disease onset in black females. METHODS: We conducted retrospective chart review of 337 patients with clinically-suspected MF seen at Johns Hopkins between 2003 and 2018, requiring biopsy-proven disease for study inclusion. Patient demographics, initial stage/percent body surface area (BSA) involvement, pathology type, flow cytometry results, and treatment regimens were recorded. RESULTS: Of 303 patients with confirmed MF, 166 (55%) were white, 107 (35%) black...
Source: Journal of the National Medical Association - Category: General Medicine Tags: J Natl Med Assoc Source Type: research
Abstract: Syringotropic mycosis fungoides (MF) is classified under folliculotropic MF. Although there is significant overlap between the 2, this study demonstrates that folliculotropism is frequently present in syringotropic MF, and when not present, the specimen did not include a follicle to examine. In addition, few of the pathology reports mentioned folliculotropism or syringotropism, although this is an important prognostic feature.
Source: The American Journal of Dermatopathology - Category: Pathology Tags: Original Study Source Type: research
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