title: Novel variant of EATL evolving from mucosal γδ-T-cells in a patient with type I RCD.
abstract: OBJECTIVE
  Enteropathy associated T-cell lymphoma (EATL) is a rare non-Hodgkin lymphoma that may complicate coeliac disease and typically occurs in patients with refractoriness to the gluten-free diet. The majority of these patients harbour a clonal expansion of intraepithelial lymphocytes (IELs) with an aberrant phenotype in the small intestine which are thus considered as the 'precursor' lymphoma cells. We describe a 51-year-old female patient with refractory coeliac disease (RCD) who developed an EATL with manifestations in the proximal small intestine and in a mesenteric lymph node that did not evolve from regular type 'aberrant' αβ-T-cells but rather from a clonal expansion of γδ-T-cells.
  
  
  METHODS
  Duodenal biopsies and lymphoma tissue from a patient with refractory coeliac disease whom developed an EATL were extensively studied by immunophenotypical, T-cell receptor immunogenetic and chromosomal analysis.
  
  
  RESULTS
  Flow cytometric analysis of duodenal IELs revealed an unusual large clonal expansion of CD30 negative γδ-T-cells in a patient with RCD. When the patient clinically deteriorated 18 months later, a substantial part (30%) of this cell population did express CD30. In addition, identical immunogenetic aberrancies had developed in a prehepatic lymph node.
  
  
  CONCLUSIONS
  We here report on a case of extraintestinal EATL that originated from a clonal γδ-IEL population rather than from aberrant IEL. This EATL displayed a distinctive pattern of immunophenotypical, T-cell receptor immunogenetic and chromosomal aberrancies as compared to classical EATL, defining this lymphoma as a novel variant of EATL.
authors.affiliation: Department of Gastroenterology , VU University Medical Center , The Netherlands.
authors.forename: R L J
authors.initials: RL
authors.lastname: van Wanrooij
authors.affiliation: Department of Pathology , VU University Medical Center , The Netherlands.
authors.forename: D
authors.initials: D
authors.lastname: de Jong
authors.affiliation: Department of Immunology , Erasmus University Medical Center , The Netherlands.
authors.forename: A W
authors.initials: AW
authors.lastname: Langerak
authors.affiliation: Department of Pathology , VU University Medical Center , The Netherlands.
authors.forename: B
authors.initials: B
authors.lastname: Ylstra
authors.affiliation: Department of Pathology , VU University Medical Center , The Netherlands.
authors.forename: H F
authors.initials: HF
authors.lastname: van Essen
authors.affiliation: Department of Pathology , VU University Medical Center , The Netherlands.
authors.forename: D A M
authors.initials: DA
authors.lastname: Heideman
authors.affiliation: Department of Pathology , VU University Medical Center , The Netherlands.
authors.forename: H J
authors.initials: HJ
authors.lastname: Bontkes
authors.affiliation: Department of Gastroenterology , VU University Medical Center , The Netherlands.
authors.forename: C J J
authors.initials: CJ
authors.lastname: Mulder
authors.affiliation: Department of Gastroenterology , VU University Medical Center , The Netherlands.
authors.forename: G
authors.initials: G
authors.lastname: Bouma
keywords: CELIAC DISEASE; INTESTINAL T CELLS; LYMPHOMA; MOLECULAR IMMUNOLOGY; T-CELL RECEPTOR