title: Enteropathy associated T cell lymphoma in celiac disease: a large retrospective study.
abstract: BACKGROUND
  Prognosis of enteropathy-associated T cell lymphoma is poor but predictors of survival remain ill-defined. How clinical presentation, pathological features and therapies influence outcome was evaluated in 37 thoroughly characterized patients with celiac disease and T-cell lymphoma.
  
  
  METHODS
  Medical files were studied retrospectively. Lymphoma and intestinal mucosa were analysed by histopathology, multiplex PCR and intestinal intraepithelial lymphocytes phenotyping. Survival and prognostic factors were analysed using Kaplan-Meier curves with Logrank test and Cox Model.
  
  
  RESULTS
  Lymphoma complicated non clonal enteropathy, celiac disease (n=15) and type I refractory celiac disease (n=2) in 17 patients and clonal type II refractory celiac disease in 20 patients. Twenty-five patients underwent surgery with resection of the main tumour mass in 22 cases. In univariate analysis, non clonal celiac disease, serum albumin level>21.6g/L at diagnosis, chemotherapy and surgical resection predicted good survival (p=0.0007, p<0.0001, p<0.0001, p<0.0001, respectively). In multivariate analysis, serum albumin level>21.6g/L, chemotherapy and reductive surgery were all significantly associated with increased survival (p<0.002, p<0.03, p<0.03, respectively).
  
  
  CONCLUSIONS
  Our study underlines the prognostic value of celiac disease type in patients with T-cell lymphoma, and suggests that a combination of nutritional, chemotherapy and reductive surgery may improve survival.
authors: Université Paris Descartes, Paris, France. georgia.malamut@egp.aphp.fr
authors: Georgia
authors: G
authors: Malamut
authors: Olivia
authors: O
authors: Chandesris
authors: Virginie
authors: V
authors: Verkarre
authors: Bertrand
authors: B
authors: Meresse
authors: Céline
authors: C
authors: Callens
authors: Elizabeth
authors: E
authors: Macintyre
authors: Yoram
authors: Y
authors: Bouhnik
authors: Jean-Marc
authors: JM
authors: Gornet
authors: Matthieu
authors: M
authors: Allez
authors: Raymond
authors: R
authors: Jian
authors: Anne
authors: A
authors: Berger
authors: Gilles
authors: G
authors: Châtellier
authors: Nicole
authors: N
authors: Brousse
authors: Olivier
authors: O
authors: Hermine
authors: Nadine
authors: N
authors: Cerf-Bensussan
authors: Christophe
authors: C
authors: Cellier
mesh_terms: D000328:Adult; D000368:Aged; D002423:Cause of Death; D002446:Celiac Disease / Q000150:complications* / Q000473:pathology; D058527:Enteropathy-Associated T-Cell Lymphoma / Q000150:complications* / Q000473:pathology; D005260:Female; D005434:Flow Cytometry; D006801:Humans; D007150:Immunohistochemistry; D007413:Intestinal Mucosa / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D016019:Survival Analysis; D016896:Treatment Outcome