Enteropathy-associated T-cell lymphoma complicating an autoimmune enteropathy

Gastroenterology. 2012 Apr;142(4):726-729.e3; quiz e13-4. doi: 10.1053/j.gastro.2011.12.040. Epub 2012 Jan 4.

Abstract

Enteropathy-associated T-cell lymphoma (EATL) is a rare non-Hodgkin lymphoma frequently associated with celiac disease. We report a case of EATL complicating adult autoimmune enteropathy (AIE). Analysis of phenotype, rearrangements in T-cell receptor genes, and chromosome alterations by high-resolution comparative genomic hybridization identified features distinct from those described for types I and II EATL. Furthermore, EATL arose from a single T-cell clone that had been present for several years in AIE-associated, oligoclonal, intestinal T-cell infiltrate. Emerging T-cell clones should be monitored in patients with AIE who receive long-term immunosuppressive therapy.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Biopsy
  • Clone Cells / immunology
  • Clone Cells / pathology
  • Comparative Genomic Hybridization
  • Enteropathy-Associated T-Cell Lymphoma / etiology*
  • Enteropathy-Associated T-Cell Lymphoma / genetics
  • Enteropathy-Associated T-Cell Lymphoma / immunology
  • Enteropathy-Associated T-Cell Lymphoma / pathology
  • Female
  • Gene Rearrangement, T-Lymphocyte
  • Genes, T-Cell Receptor*
  • Genetic Predisposition to Disease
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Intestinal Neoplasms / etiology*
  • Intestinal Neoplasms / genetics
  • Intestinal Neoplasms / immunology
  • Intestinal Neoplasms / pathology
  • Middle Aged
  • Phenotype
  • Polyendocrinopathies, Autoimmune / complications*
  • Polyendocrinopathies, Autoimmune / drug therapy
  • Polyendocrinopathies, Autoimmune / immunology

Substances

  • Immunosuppressive Agents

Supplementary concepts

  • Autoimmune enteropathy