Expression of CCR3 and CCR4 Suggests a Poor Prognosis in Mycosis Fungoides and S ézary Syndrome.

Expression of CCR3 and CCR4 Suggests a Poor Prognosis in Mycosis Fungoides and Sézary Syndrome. Acta Derm Venereol. 2019 May 02;: Authors: Shono Y, Suga H, Kamijo H, Fujii H, Oka T, Miyagaki T, Shishido-Takahashi N, Sugaya M, Sato S Abstract Tumor cells in cutaneous T cell lymphoma express limited numbers of chemokine receptors. We investigated the expression patterns of CXCR3, CCR3, CCR4 and CCR10 in mycosis fungoides, Sézary syndrome, lym-phomatoid papulosis and anaplastic large cell lymphoma in 121 skin biopsy samples. CXCR3 was expressed in 86% of mycosis fungoides cases but in no anaplastic large cell lymphoma cases. CCR3 was expressed in 73% of cases of CD30+ lymphoproliferative disorders such as lymphomatoid papulosis and anaplastic large cell lymphoma. Mycosis fungoides/Sézary syndrome patients with high CCR3 or CCR4 expression had a poorer survival prognosis than mycosis fungoides/Sézary syndrome patients whose tumor cells did not express these receptors. CCR10 was expressed in 50% of mycosis fungoides/Sézary syndrome cases and in 13% of cases with CD30+ lym-phoproliferative disorders. These results suggest that differential patterns of CXCR3, CCR3, CCR4 and CCR10 expression are useful for the diagnosis of cutaneous T cell lymphoma. Moreover, expression of CCR3 or CCR4 suggests a poor prognosis in mycosis fungoides/Sézary syndrome. PMID: 31045236 [PubMed - as supplied by publisher]
Source: Acta Dermato-Venereologica - Category: Dermatology Authors: Tags: Acta Derm Venereol Source Type: research

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We report here that low-dose radiation therapy (LDRT, 8 Gy) induced durable remissions of treated lesions. We hypothesized that treatment with LDRT may era dicate the malignant T cell clone within the treated site. To address this, we examined pre- and post-treatment biopsies from 20 lesional skin samples of 18 MF patients who received either 8 Gy LDRT (n=16) or topical steroids (n=4) with high-throughput T-cell receptor sequencing (HTS) of the TCRB ge ne to identify, quantify, and follow the malignant T cell clone.
Source: Journal of Investigative Dermatology - Category: Dermatology Authors: Tags: Translational Studies Source Type: research
Conclusions Epidemiological studies have repeatedly helped identify definitive triggers for several diseases. As highlighted in this perspective report, previous studies strongly argue for the interplay between intrinsic factors and putative preventable extrinsic triggers/promoters for CTCL. Given the evidence of geographical regional clustering of CTCL patients, CTCL occurrence in unrelated family members and recent evidence implicating S. aureus in the pathogenesis/progression of CTCL, more research is needed to decipher the precise mechanism by which specific environmental exposures may be driving the pathogenesis of t...
Source: Frontiers in Oncology - Category: Cancer & Oncology Source Type: research
CONCLUSIONS: These findings can be explained by a model where malignant transformation takes place during early T-cell development giving rise to circulating pre-malignant clones which home to the skin producing clinically apparent lesions of cutaneous lymphoma. Therapeutic strategies in T-cell lymphoma should therefore target those early lymphoma precursor cells. PMID: 30808775 [PubMed - as supplied by publisher]
Source: Clinical Cancer Research - Category: Cancer & Oncology Authors: Tags: Clin Cancer Res Source Type: research
A 34-year-old Japanese woman presented with widespread scaly erythema that had enlarged over 2 years. A skin biopsy revealed the diagnosis of mycosis fungoides (patch stage, T1b N0 M0 B0), a most frequent cutaneous T-cell lymphoma. 18F-FDG PET/CT scan unexpectedly showed intense uptake on the left sole, which suggested a tumorous mycosis fungoides lesion (SUVmax = 6.2). Careful examination revealed the mass to be a typical plantar wart of 2 cm in diameter that the patient had not recognized. With repeated cryotherapy, the wart disappeared in 6 months, and follow-up 18F-FDG PET/CT showed no abnormal uptake on the left sole.
Source: Clinical Nuclear Medicine - Category: Nuclear Medicine Tags: Interesting Images Source Type: research
Conclusions: Durvalumab/lenalidomide has significant clinical activity in patients with refractory/advanced CTCL, which will be formally evaluated in the Phase 2 portion of this trial. Responses were durable and ongoing, and treatment was well tolerated with a low toxicity profile. Dose escalation is planned up to lenalidomide 20 mg daily. Our preliminary results from patients on trial demonstrated that immune signatures on skin biopsies at baseline may be predictive of response to checkpoint blockade and yield insights into mechanisms of therapeutic resistance.DisclosuresQuerfeld: Acelion: Membership on an entity's Board ...
Source: Blood - Category: Hematology Authors: Tags: 624. Hodgkin Lymphoma and T/NK Cell Lymphoma-Clinical Studies: Poster II Source Type: research
Cutaneous T-cell lymphomas (CTCL) represent up to 80% of extranodal non-Hodgkin Lymphomas, the most common being Mycosis Fungoides (MF) with or without its leukemic stage Sezary Syndrome and Primary cutaneous CD30+ T-cell lymphoproliferative disorders. Prognosis for advanced stages is poor, with 5-year survival 40-70% for patients with advanced skin stages, and 15-40% for those with extracutaneous involvement and CD30+ transformed CTCL. The disease is considered incurable, and most patients will undergo at least two different lines of therapy, and up to 36% undergo at least four different lines due to a short duration of r...
Source: Blood - Category: Hematology Authors: Tags: 624. Hodgkin Lymphoma and T/NK Cell Lymphoma-Clinical Studies Source Type: research
Conclusions: Lymphoma cells in MF/SS show marked heterogeneity of expression of PD-1 including clear subset arising from TFH. This suggests MF/SS may represent multiple biological entities. Further studies will be necessary to investigate the clinical significance of cell-of-origin of MF/SS.DisclosuresYabe: Y-mAbs Therapeutics: Consultancy. Moskowitz: ADC Therapeutics: Research Funding; Incyte: Research Funding; Bristol Myers-Squibb: Consultancy, Research Funding; Takeda: Honoraria; Merck: Research Funding; Seattle Genetics: Consultancy, Honoraria, Research Funding. Horwitz: Infinity/Verastem: Consultancy, Research Funding...
Source: Blood - Category: Hematology Authors: Tags: 624. Hodgkin Lymphoma and T/NK Cell Lymphoma-Clinical Studies: Poster I Source Type: research
Abstract: Cutaneous peripheral T-cell lymphoma, not otherwise specified represents a “waste basket” of all cases that cannot be put into another of the categories of mature cutaneous T-cell lymphoma. Previously, the sudden multifocal development of cutaneous CD4+ tumors without preceding a patch or plaque stage was classified as d'emblée form of mycosis fungoides (MF). Currently, the term “MF” reserved only for the classic Alibert–Bazin type characterized by the evolution of patches, plaques, and tumors or for variants showing a similar clinical course. The authors describe a 75-year-...
Source: The American Journal of Dermatopathology - Category: Pathology Tags: Extraordinary Case Report Source Type: research
Mycosis fungoides (MF) is one of the most common types of extranodal T-cell lymphomas and is considered to be caused by malignant transformation of the mature T-cell residing in the skin. Since T-cell receptor (TCR) genes γ, β and α are sequentially rearranged during thymic T-cell development but not in mature T-cells, it can be predicted that all malignant cells in MF share identical TCR-gene sequences. Here we have performed a detailed analysis of TCR rearrangements in biopsies from MF tumors composed of>80% of malignant cells using next-generation sequencing (NGS).
Source: European Journal of Cancer - Category: Cancer & Oncology Authors: Source Type: research
Mycosis fungoides (MF), the most common cutaneous T cell lymphoma (CTCL) is a malignancy of skin-tropic memory T cells. Most MF cases present as early stage (stage I A/B, limited to the skin), and these patients typically have a chronic, indolent clinical course. However, a small subset of early-stage cases develop progressive and fatal disease. Because outcomes can be so different, early identification of this high-risk population is an urgent unmet clinical need. We evaluated the use of next-generation high-throughput DNA sequencing of the T cell receptor β gene (TCRB) in lesional skin biopsies to predict progressio...
Source: Science Translational Medicine - Category: Biomedical Science Authors: Tags: Research Articles Source Type: research
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