title: High frequency of fatal haemophagocytic lymphohistiocytosis syndrome in enteropathy-associated T cell lymphoma.
abstract: BACKGROUND
  Enteropathy-associated T-cell lymphoma is a rare form of T-cell lymphoma associated with a poor prognosis and the relative ineffectiveness of standard chemotherapy. The occurrence of haemophagocytic lymphohistiocytosis has been reported only once with this entity.
  
  
  METHODS
  A retrospective study of 15 patients with enteropathy-associated T-cell lymphoma (type 1 in 12), followed-up in our units, since 1985. Two patients died before starting chemotherapy. The remaining 13 patients were treated with standard chemotherapy (n=7) and purine nucleotide analogues (n=6).
  
  
  RESULTS
  Median follow-up was 8.7 (1-97) months. Surgery was required in 10 patients (66%) for intestinal complications (n=7) or elective small bowel resection (n=3). Survival probability was 40% and 20% at 1 and 5 years, respectively (Kaplan-Meier method). Survival was not significantly different between the two chemotherapy regimens. However, a slight decrease of febrile neutropenia was observed in the purine nucleotide analogues group (p=0.06). Haemophagocytic lymphohistiocytosis occurred in 6/15 (40%) cases. In these six patients, haemophagocytic lymphohistiocytosis was always fatal within 3 months.
  
  
  CONCLUSIONS
  Enteropathy-associated T-cell lymphoma is associated with a poor outcome, independently of the chemotherapy regimens administered and frequent occurrence of haemophagocytic lymphohistiocytosis. The latter complication should be considered for urgent rescue therapy.
authors.affiliation: Pôle des Maladies de l'Appareil Digestif, Service de Gastroentérologie et d'Assistance Nutritive, Hôpital Beaujon, Clichy et Université Diderot, Paris VII, France. aurelien.amiot@bjn.aphp.fr
authors.forename: Aurelien
authors.initials: A
authors.lastname: Amiot
authors.forename: Matthieu
authors.initials: M
authors.lastname: Allez
authors.forename: Xavier
authors.initials: X
authors.lastname: Treton
authors.forename: Claire
authors.initials: C
authors.lastname: Fieschi
authors.forename: Lionel
authors.initials: L
authors.lastname: Galicier
authors.forename: Francisca
authors.initials: F
authors.lastname: Joly
authors.forename: Jean-Marc
authors.initials: JM
authors.lastname: Gornet
authors.forename: Eric
authors.initials: E
authors.lastname: Oksenhendler
authors.forename: Marc
authors.initials: M
authors.lastname: Lémann
authors.forename: Yoram
authors.initials: Y
authors.lastname: Bouhnik
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000009:adverse effects / Q000627:therapeutic use*; D017338:Cladribine / Q000008:administration & dosage; D018450:Disease Progression; D018572:Disease-Free Survival; D058527:Enteropathy-Associated T-Cell Lymphoma / Q000150:complications / Q000188:drug therapy* / Q000601:surgery; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications / Q000188:drug therapy* / Q000601:surgery; D008297:Male; D008875:Middle Aged; D009503:Neutropenia / Q000209:etiology; D011685:Purine Nucleotides / Q000009:adverse effects / Q000627:therapeutic use*; D012189:Retrospective Studies
chemical_list: D011685:Purine Nucleotides; D017338:Cladribine