title: Abnormal intestinal intraepithelial lymphocytes in refractory sprue.
abstract: OBJECTIVE
  The etiology of refractory sprue is unclear. To gain insight into its pathogenesis, the phenotype and T-cell receptor (TCR) gene rearrangement status of intestinal lymphocytes were analyzed in a group of patients with clinical or biological features of celiac disease but either initially or subsequently refractory to a gluten-free diet.
  
  
  METHODS
  Intestinal biopsy specimens were obtained from 26 adults: 6 patients with refractory sprue, 7 patients with active celiac disease, and 13 normal controls. The phenotype of intestinal lymphocytes was studied by immunohistochemistry and, in 3 patients with refractory sprue, by cytometry of lymphocytes purified from intestinal biopsy specimens. TCR rearrangements were assessed by studying TCRgammaV-J junctional regions from DNA extracted from intestinal biopsy specimens and purified intestinal lymphocytes.
  
  
  RESULTS
  In the 6 patients with refractory sprue, but not in normal controls or patients with active celiac disease, the intestinal epithelium was massively infiltrated by small lymphocytes that lacked CD8, CD4, and TCR, contained intracytoplasmic but not surface CD3epsilon chains, and exhibited restricted TCRgamma gene rearrangements.
  
  
  CONCLUSIONS
  Refractory sprue is associated with an abnormal subset of intraepithelial lymphocytes containing CD3epsilon and restricted rearrangements of the TCRgamma chain but lacking surface expression of T-cell receptors.
authors.affiliation: Department of Gastroenterology, Hôpital Laënnec, Paris, France.
authors.forename: C
authors.initials: C
authors.lastname: Cellier
authors.forename: N
authors.initials: N
authors.lastname: Patey
authors.forename: L
authors.initials: L
authors.lastname: Mauvieux
authors.forename: B
authors.initials: B
authors.lastname: Jabri
authors.forename: E
authors.initials: E
authors.lastname: Delabesse
authors.forename: J P
authors.initials: JP
authors.lastname: Cervoni
authors.forename: M L
authors.initials: ML
authors.lastname: Burtin
authors.forename: D
authors.initials: D
authors.lastname: Guy-Grand
authors.forename: Y
authors.initials: Y
authors.lastname: Bouhnik
authors.forename: R
authors.initials: R
authors.lastname: Modigliani
authors.forename: J P
authors.initials: JP
authors.lastname: Barbier
authors.forename: E
authors.initials: E
authors.lastname: Macintyre
authors.forename: N
authors.initials: N
authors.lastname: Brousse
authors.forename: N
authors.initials: N
authors.lastname: Cerf-Bensussan
mesh_terms: D000328:Adult; D000368:Aged; D015703:Antigens, CD / Q000032:analysis; D002446:Celiac Disease / Q000209:etiology / Q000276:immunology* / Q000473:pathology; D005260:Female; D015334:Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor; D006801:Humans; D016130:Immunophenotyping; D007422:Intestines / Q000276:immunology*; D008875:Middle Aged; D016133:Polymerase Chain Reaction; D016176:T-Lymphocyte Subsets / Q000276:immunology*
chemical_list: D015703:Antigens, CD