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Original article
Specific TCR gene rearrangements in mycosis fungoides: does advanced clinical stage show a preference?
  1. Etan Marks1,
  2. Yanhua Wang2,
  3. Yang Shi2,
  4. Joseph Susa3,
  5. Mark Jacobson2,
  6. D Yitzchak Goldstein2
  1. 1 Department of Pathology, NYU Langone Medical Center, New York, USA
  2. 2 Department of Pathology, Montefiore Medical Center/Albert Einstein College of Medicine, Bronx, New York, USA
  3. 3 Division of Dermatopathology, Cockerell Dermatopathology, Dallas, Texas, USA
  1. Correspondence to Dr Etan Marks, NYU Langone Medical Center, NY 10016, USA; et565{at}aol.com

Abstract

Aims The relationship between the presence of specific T-cell receptor (TCR) gene rearrangements and clinical stage in mycosis fungoides (MF) has not been studied. We analysed a cohort of patients with a diagnosis of MF to determine the different types of specific TCR gene rearrangements present and their relationship to disease stage.

Methods A retrospective chart review was used to select patients with a diagnosis of MF who had a skin biopsy and a positive TCR gene rearrangement study in either blood or tissue and at least 2 years of clinical follow-up.

Results 43 patients were identified and divided into two groups. The first group consisted of 23 patients with early stage disease (IA-IIA) that was either stable or went into partial or complete remission with minimal intervention. None of these patients advanced to late stage disease. The second group consisted of 20 patients who had either late stage disease at diagnosis or progressed to late stage disease at some point in time. In the first group, only 4/23 (17%) patients had a single TCR gene rearrangement in the Vɣ1–8 region. In contrast, the second group had 13/20 (65%) patients with a single TCR gene rearrangement in the Vɣ1–8 region (p=0.002).

Conclusion The presence of a single TCR gene rearrangement in the Vɣ1–8 region could possibly be related to a more advanced stage of MF. However, more comprehensive studies, such as next generation sequencing, with a larger cohort is necessary for a more definitive conclusion.

  • TCR
  • Gene rearrangement
  • T-cell lymphoma
  • Mycosis fungoides
  • Vg1-8

https://doi.org/10.1136/jclinpath-2018-205324

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    Footnotes

    • Handling editor Mary Frances McMullin.

    • Contributors All authors substantially contributed to the conception, design of the work as well as the acquisition, analysis and interpretation of data. All authors helped draft the work as well as revising it. All authors gave final approval of the version currently being submitted. All authors agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Patient consent Not required.

    • Ethics approval Montefiore/Albert Einstein College of Medicine.

    • Provenance and peer review Not commissioned; externally peer reviewed.

    • Data sharing statement There are no available unpublished data from the study.