title: Enteropathy-associated T-cell lymphoma: clinical and histological findings from the international peripheral T-cell lymphoma project.
abstract: Few large, international series of enteropathy-associated T-cell lymphoma (EATL) have been reported. We studied a cohort of 62 patients with EATL among 1153 patients with peripheral T-cell or natural killer (NK)-cell lymphoma from 22 centers worldwide. The diagnosis was made by a consensus panel of 4 expert hematopathologists using World Health Organization (WHO) criteria. Clinical correlations and survival analyses were performed. EATL comprised 5.4% of all lymphomas in the study and was most common in Europe (9.1%), followed by North America (5.8%) and Asia (1.9%). EATL type 1 was more common (66%) than type 2 (34%), and was especially frequent in Europe (79%). A clinical diagnosis of celiac sprue was made in 32.2% of the patients and was associated with both EATL type 1 and type 2. The median overall survival was only 10 months, and the median failure-free survival was only 6 months. The International Prognostic Index (IPI) was not as good a predictor of survival as the Prognostic Index for Peripheral T-Cell Lymphoma (PIT). Clinical sprue predicted for adverse survival independently of the PIT. Neither EATL subtype nor other biologic parameters accurately predicted survival. Our study confirms the poor prognosis of patients with EATL and the need for improved treatment options.
authors.affiliation: Departments of Pathology, University Hospital of Oslo, Oslo, Norway. jan.delabie@medisin.uio.no
authors.forename: Jan
authors.initials: J
authors.lastname: Delabie
authors.forename: Harald
authors.initials: H
authors.lastname: Holte
authors.forename: Julie M
authors.initials: JM
authors.lastname: Vose
authors.forename: Fred
authors.initials: F
authors.lastname: Ullrich
authors.forename: Elaine S
authors.initials: ES
authors.lastname: Jaffe
authors.forename: Kerry J
authors.initials: KJ
authors.lastname: Savage
authors.forename: Joseph M
authors.initials: JM
authors.lastname: Connors
authors.forename: Lisa
authors.initials: L
authors.lastname: Rimsza
authors.forename: Nancy L
authors.initials: NL
authors.lastname: Harris
authors.forename: Konrad
authors.initials: K
authors.lastname: Müller-Hermelink
authors.forename: Thomas
authors.initials: T
authors.lastname: Rüdiger
authors.forename: Bertrand
authors.initials: B
authors.lastname: Coiffier
authors.forename: Randy D
authors.initials: RD
authors.lastname: Gascoyne
authors.forename: Françoise
authors.initials: F
authors.lastname: Berger
authors.forename: Kensei
authors.initials: K
authors.lastname: Tobinai
authors.forename: Wing Y
authors.initials: WY
authors.lastname: Au
authors.forename: Raymond
authors.initials: R
authors.lastname: Liang
authors.forename: Emili
authors.initials: E
authors.lastname: Montserrat
authors.forename: Ephraim P
authors.initials: EP
authors.lastname: Hochberg
authors.forename: Stefano
authors.initials: S
authors.lastname: Pileri
authors.forename: Massimo
authors.initials: M
authors.lastname: Federico
authors.forename: Bharat
authors.initials: B
authors.lastname: Nathwani
authors.forename: James O
authors.initials: JO
authors.lastname: Armitage
authors.forename: Dennis D
authors.initials: DD
authors.lastname: Weisenburger
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002446:Celiac Disease / Q000145:classification / Q000401:mortality* / Q000473:pathology*; D015331:Cohort Studies; D032921:Consensus; D058527:Enteropathy-Associated T-Cell Lymphoma / Q000145:classification / Q000401:mortality* / Q000473:pathology*; D005260:Female; D006801:Humans; D038622:Internationality; D007694:Killer Cells, Natural / Q000473:pathology; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D011379:Prognosis; D016019:Survival Analysis; D013601:T-Lymphocytes / Q000473:pathology; D014944:World Health Organization