Spontaneously relapsing clonal, mucosal cytotoxic T-cell lymphoproliferative disorder: case report and review of the literature

Am J Surg Pathol. 2000 Feb;24(2):296-301. doi: 10.1097/00000478-200002000-00017.

Abstract

Primary T-cell lymphoma of the gastrointestinal tract is a rare and usually aggressive disorder that may be associated with celiac disease. The authors describe a unique case of a clonal proliferation of CD8+ T cells involving the oral mucosa, ileum, and colon of a 35-year-old man that has regressed spontaneously and recurred numerous times over a 9-year period without treatment. The patient's symptoms were limited to occasional rectal bleeding and recurring painful oral ulcers. Within the intestine, these collections of small T cells induced minimal architectural distortions and did not show extensive epitheliotrophism. Polymerase chain reaction and sequencing analyses revealed that the identical T-cell clone has been present for more than 9 years and in different mucosal locations in this patient. This may represent a unique T-cell lymphoproliferative process akin to a mucosal counterpart of lymphomatoid papulosis of the skin.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Base Sequence
  • Biomarkers, Tumor / analysis
  • Clone Cells
  • DNA Primers / chemistry
  • DNA, Neoplasm / analysis
  • Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor / genetics
  • Humans
  • Immunoenzyme Techniques
  • Intestinal Mucosa / pathology*
  • Intestinal Neoplasms / chemistry
  • Intestinal Neoplasms / genetics
  • Intestinal Neoplasms / pathology*
  • Lymphoma, T-Cell / chemistry
  • Lymphoma, T-Cell / genetics
  • Lymphoma, T-Cell / pathology*
  • Male
  • Molecular Sequence Data
  • Mouth Mucosa / pathology*
  • Mouth Neoplasms / chemistry
  • Mouth Neoplasms / genetics
  • Mouth Neoplasms / pathology*
  • Neoplasm Regression, Spontaneous / genetics
  • Neoplasm Regression, Spontaneous / pathology*
  • Polymerase Chain Reaction
  • Receptors, Antigen, T-Cell, alpha-beta / genetics
  • T-Lymphocytes, Cytotoxic / pathology*

Substances

  • Biomarkers, Tumor
  • DNA Primers
  • DNA, Neoplasm
  • Receptors, Antigen, T-Cell, alpha-beta