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A New and Validated Clinical Prognostic Model (EPI) for Enteropathy-Associated T-cell Lymphoma

Lookup NU author(s): Dr Michal Sieniawski, Dr Anne Lennard


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Purpose: Enteropathy-associated T-cell lymphoma (EATL) is a rare intestinal non-Hodgkin lymphoma with a poor, though variable prognosis. The International Prognostic Index (IPI) and the prognostic index for peripheral T-cell lymphoma (PIT) have limited predictive value for outcome of EATL. The purpose of this studywas to develop and validate a prognostic model for EATL, which can identify high-risk patients who need more aggressive therapy.Experimental Design: This retrospective multicenter study was based on 92 patients and included 45 patients diagnosed with EATL between 1999 and 2009 from the Netherlands and 47 patients from England and Scotland, diagnosed with EATL between 1994 and 1998. A new EATL prognostic index (EPI) was constructed using the RPART (recursive partitioning and regression trees) procedure. Validation was performed applying the bootstrap method.Results: Three risk groups were distinguished (P < 0.0001): a high-risk group, characterized by the presence of B-symptoms [median overall survival (OS) of 2 months]; an intermediate-risk group, comprising patients without B-symptoms and an IPI score >= 2 (7 months); and a low-risk group, representing patients without B- symptoms and an IPI score of 0 to 1 (34 months). Internal validation showed stability of statistical significance and prognostic discrimination. In contrast with the IPI and PIT, the EPI better classified patients in risk groups according to their clinical outcome.Conclusions: Our new, validated, prognostic model EPI accurately predicts survival outcome in EATL and may be used for patient selection for new therapeutic strategies and evaluation of clinical trials. (C) 2015 AACR.

Publication metadata

Author(s): de Baaij LR, Berkhof J, van de Water JMW, Sieniawski MK, Radersma M, Verbeek WHM, Visser OJ, Oudejans JJ, Meijer CJLM, Mulder CJJ, Lennard AL, Cillessen SAGM

Publication type: Article

Publication status: Published

Journal: Clinical Cancer Research

Year: 2015

Volume: 21

Issue: 13

Pages: 3013-3019

Print publication date: 01/07/2015

Online publication date: 16/03/2015

Acceptance date: 01/01/1900

ISSN (print): 1078-0432

ISSN (electronic): 1557-3265

Publisher: American Association for Cancer Research


DOI: 10.1158/1078-0432.CCR-14-2195


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Funder referenceFunder name
PV06/18MDLStichting VUmc Cancer Center Amsterdam
WO-09-28Dutch Digestive foundation