You are here

Article Text

Article menu
Download PDFPDF
Coeliac disease
Refractory coeliac sprue is a diffuse gastrointestinal disease
  1. V Verkarre1,
  2. V Asnafi3,
  3. T Lecomte6,
  4. N Patey Mariaud-De Serre2,
  5. M Leborgne2,
  6. E Grosdidier6,
  7. C Le Bihan4,
  8. E Macintyre3,
  9. C Cellier6,
  10. N Cerf-Bensussan5,
  11. N Brousse2
  1. 1Department of Pathology and Université René Descartes-Paris V (EA219), AP-HP, Hôpital Necker-Enfants Malades, 75015 Paris, France, and INSERM EMI-0212, Faculté Necker-Paris V, 75015 Paris, France
  2. 2Department of Pathology and Université René Descartes-Paris V (EA219), AP-HP, Hôpital Necker-Enfants Malades, 75015 Paris, France
  3. 3Department of Biological Hematology, AP-HP, Hôpital Necker-Enfants Malades, 75015 Paris, France
  4. 4Department of Biostatistics, AP-HP, Hôpital Necker-Enfants Malades, 75015 Paris, France
  5. 5INSERM EMI-0212, Faculté Necker-Paris V, 75015 Paris, France
  6. 6Department of Gastroenterology, AP-HP, Hôpital Européen Georges Pompidou, 75015 Paris, France
  1. Correspondence to:
    Dr V Verkarre, Service d’Anatomie et de Cytologie Pathologiques, Hôpital Necker-Enfants Malades, 149 rue de Sévres, 75743 Paris Cedex 15, France;
    virginie.verkarre{at}nck.ap-hop-paris.fr

Abstract

Background: Refractory coeliac sprue (RCS) with an immunophenotypically aberrant clonal intraepithelial lymphocyte (IEL) population is considered a cryptic form of intestinal T cell lymphoma.

Aims: To investigate the distribution of the abnormal and monoclonal IEL population in the digestive tract of RCS patients.

Patients and methods: We compared the frequency of lymphocytic gastritis (LG) and lymphocytic colitis (LC), together with IEL phenotype and T cell clonality, in gastric and colonic samples from 15 adults with RCS (all with aberrant CD3 intracytoplasmic+ surface− CD8− clonal IELs on duodenojejunal biopsies), 18 patients with active coeliac disease (ACD), and 10 patients with coeliac disease (CD) on a gluten free diet (GFD-CD) by means of immunohistochemistry and multiplex polymerase chain reaction amplification of the T cell receptor γ gene (TCR-γ) rearrangement. Blood samples of nine RCS patients were also tested for clonality.

Results: LG was found in 9/14 (64%), 11/18 (61%), and 3/10 (30%) patients with RCS, ACD, and GFD-CD, respectively, while LC was found in 6/11 (55%), 3/4 (75%), and 2/3 (66%) patients. Contrary to CD, all samples from patients with LG and LC showed an aberrant IEL phenotype. Monoclonal TCR-γ rearrangements were detected in 8/13 (62%), 8/10 (80%), and 4/9 (44%) of gastric, colonic, and blood samples, respectively, from RCS patients, while in CD patients such rearrangements were only found in 2/25 (8%) gastric samples.

Conclusion: The immunophenotypically aberrant monoclonal IEL population present in the small intestine of patients with RCS frequently disseminates to the blood and the entire gastrointestinal epithelium, suggesting that this is a diffuse gastrointestinal disease.

  • enteropathy-type intestinal T cell lymphoma
  • lymphocytic gastritis
  • lymphocytic colitis
  • coeliac disease
  • refractory sprue
  • T cell receptor gene rearrangement
  • ACD, active coeliac disease
  • CD, coeliac disease
  • GFD, gluten free diet
  • GFD-CD, CD on a GFD
  • EITCL, enteropathy-type intestinal T cell lymphoma
  • IEL, intraepithelial lymphocyte
  • LC, lymphocytic colitis
  • LG, lymphocytic gastritis
  • PCR, polymerase chain reaction
  • RCS, refractory coeliac sprue
  • TCR, T cell receptor

https://doi.org/10.1136/gut.52.2.205

Statistics from Altmetric.com

    Request Permissions

    If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.

    View Full Text

    Footnotes

    Linked Articles