Analysis of β, γ, and δ T-Cell receptor genes in mycosis fungoides and sezary syndrome
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TLDR
Results indicate that in mycosis fungoides (MF) T‐cell clones can be detected in skin biopsy specimens from the majority of patients with early stage disease, γδ T-cell clones are only rarely found, and TCR gene analysis can detect T‐ cell clones in the peripheral blood with a greater degree of specificity than conventional light microscopic study.Abstract:
The authors have analyzed the configuration of immunoglobulin (Ig) and beta, gamma and delta T-cell receptor (TCR) genes in DNA extracted from skin, lymph nodes, and peripheral blood mononuclear cells obtained from 41 patients with mycosis fungoides (MF), 14 patients with Sezary syndrome, and 13 patients with benign inflammatory dermatoses. No discrete rearranged bands (DRB) were detected in patients with inflammatory dermatoses. In tissue DNA from 19 patients with MF DRB were detected with beta and gamma, but not delta TCR probes. Only one patient with MF had a rearrangement of gamma and delta with germ line beta TCR genes. In 13 patients multiple biopsies were analyzed and DRB, when present, were identical in different lesions from individual patients. In three patients analysis of DNA from dermatopathic lymph nodes did not reveal DRB. Analysis of peripheral blood DNA from 24 patients revealed a discrete rearrangement of the gamma TCR gene in four patients and both beta and gamma genes in four additional patients. In MF DRB were detected more frequently with advancing stage of disease in tissues (P less than 0.01) but not in peripheral blood (P equals 0.36). Of 14 patients with Sezary syndrome, eight had DRB in peripheral blood DNA with both beta and gamma probes and in three of these patients identical DRB were also detected in DNA from skin biopsy samples. In contrast, DRB were not detected in the peripheral blood of the other six patients. In both MF and Sezary syndrome there was no restricted usage of particular V gamma genes. These results indicate that in MF (1) T-cell clones can be detected in skin biopsy specimens from the majority of patients with early stage disease, (2) gamma delta T-cell clones are only rarely found, and (3) TCR gene analysis can detect T-cell clones in the peripheral blood with a greater degree of specificity than conventional light microscopic study. In Sezary syndrome these studies also suggest that a subset of patients have a polyclonal population of circulating atypical lymphoid cells. In addition these patients appear to have a better prognosis than those with monoclonal disease.read more
Citations
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Survival Outcomes and Prognostic Factors in Mycosis Fungoides/Sézary Syndrome: Validation of the Revised International Society for Cutaneous Lymphomas/European Organisation for Research and Treatment of Cancer Staging Proposal
Nita Agar,Emma Wedgeworth,Siobhan Crichton,Tracey J. Mitchell,Michael E. Cox,Silvia Ferreira,Alistair Robson,Eduardo Calonje,Catherine M. Stefanato,Elizabeth Mary Wain,Bridget S. Wilkins,Paul Fields,Alan Dean,Katherine Webb,Julia Scarisbrick,Stephen Morris,Sean Whittaker +16 more
TL;DR: This study validated the recently proposed ISCL/EORTC staging system and identified new prognostic factors in mycosis fungoides and Sézary syndrome patients.
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Update on erythrodermic cutaneous T-cell lymphoma: report of the International Society for Cutaneous Lymphomas.
Eric C. Vonderheid,Maria Grazia Bernengo,Günter Burg,Madeleine Duvic,Peter Heald,Liliane Laroche,Elise A. Olsen,Mark R. Pittelkow,Robin Russell-Jones,Masahiro Takigawa,Rein Willemze +10 more
TL;DR: The hematologic criteria recommended for Sézary syndrome are intended to identify patients with a worse prognosis compared with the other E-CTCL subsets and consist of one or more of the following: an absolute SéZary cell count and a CD4/CD8 ratio of 10 or higher.
Journal ArticleDOI
Primary cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome): part I. Diagnosis: clinical and histopathologic features and new molecular and biologic markers
Sarah I. Jawed,Patricia L. Myskowski,Steven M. Horwitz,Alison J. Moskowitz,Christiane Querfeld +4 more
TL;DR: The skin microenvironment, including immune cells, such as dendritic cells and reactive cytotoxic and regulatory T cells, plays a crucial supporting role in mycosis fungoides (MF) and Sezary syndrome (SS) as mentioned in this paper.
Journal ArticleDOI
Granulomatous Mycosis Fungoides and Granulomatous Slack Skin: A Multicenter Study of the Cutaneous Lymphoma Histopathology Task Force Group of the European Organization for Research and Treatment of Cancer (EORTC)
Werner Kempf,Sonja Ostheeren-Michaelis,Marco Paulli,Marco Lucioni,Janine Wechsler,Heike Audring,Chalid Assaf,Thomas Rüdiger,Rein Willemze,Chris J.L.M. Meijer,Emilio Berti,Lorenzo Cerroni,Marco Santucci,Christian Hallermann,M. Berneburg,Sergio Chimenti,Alistair Robson,Márta Marschalkó,Dmitry V. Kazakov,Tony Petrella,Sylvie Fraitag,Agnès Carlotti,Philippe Courville,Hubert R. Laeng,Robert Knobler,Philippa Golling,Reinhard Dummer,Günter Burg +27 more
TL;DR: There are clinical differences between GMF and GSS, but they show overlapping histologic findings and therefore cannot be discriminated by histologic examination alone, and the prognosis of GMF appears worse than that of classic nongranulomatous mycosis fungoides.
Journal ArticleDOI
Molecular cytogenetic analysis of cutaneous T-cell lymphomas: identification of common genetic alterations in Sézary syndrome and mycosis fungoides.
Xin Mao,Debra M. Lillington,Julia J. Scarisbrick,Tracey J. Mitchell,B. Czepulkowski,Robin Russell-Jones,Bryan D. Young,Sean Whittaker +7 more
TL;DR: This data indicates that genome‐wide surveys for chromosome aberrations in primary cutaneous T‐cell lymphoma (CTCL) are limited and further research is needed to assess the importance of these surveys for informing treatment decisions.
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