Enteropathy-associated T-cell lymphomas have a cytotoxic T-cell phenotype

Histopathology. 1997 Oct;31(4):313-7. doi: 10.1046/j.1365-2559.1997.2660862.x.


Aims: Enteropathy-associated T-cell lymphoma (EATCL) is a rare complication of coeliac disease. We investigated whether EATCLs are the neoplastic counterparts of activated cytotoxic T-cells (CTLs).

Methods and results: Eight cases, clinically and histologically defined, were stained with monoclonal antibodies against components of the cytotoxic granules of CTLs, granzyme B and T-cell restricted intracellular antigen (TIA-1). It was found that all cases had a cytotoxic phenotype, i.e. expression of TIA-1 in most of the tumour cells, whereas granzyme B was found in six of eight cases, mostly in a smaller number of tumour cells compared to TIA-1. Since TIA-1 and granzyme B are expressed at different stages of activation of CTLs it is hypothesized that differences in expression between granzyme B and TIA-1 in EATCL represent different stages of activation in which the tumour cells are arrested. Clinically, seven of the eight patients died within 10 months after diagnosis of EATCL.

Conclusions: EATCL is a clinicopathological entity with a grim prognosis and with tumour cells representing a unique neoplastic equivalent of CTLs arrested in varying stages of activation.

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Antigens, CD / metabolism
  • Biomarkers, Tumor / metabolism
  • Celiac Disease / complications
  • Celiac Disease / metabolism
  • Celiac Disease / pathology*
  • Female
  • Granzymes
  • Humans
  • Immunohistochemistry
  • Immunophenotyping
  • Lymphoma, T-Cell / complications
  • Lymphoma, T-Cell / metabolism
  • Lymphoma, T-Cell / pathology*
  • Male
  • Membrane Proteins / metabolism
  • Middle Aged
  • Poly(A)-Binding Proteins
  • Proteins*
  • RNA-Binding Proteins / metabolism
  • Serine Endopeptidases / metabolism
  • T-Cell Intracellular Antigen-1
  • T-Lymphocytes, Cytotoxic / metabolism
  • T-Lymphocytes, Cytotoxic / pathology*


  • Antigens, CD
  • Biomarkers, Tumor
  • Membrane Proteins
  • Poly(A)-Binding Proteins
  • Proteins
  • RNA-Binding Proteins
  • T-Cell Intracellular Antigen-1
  • TIA1 protein, human
  • GZMB protein, human
  • Granzymes
  • Serine Endopeptidases