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

Abstract

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 study was 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.

T- and NK-cell lymphoproliferative disorders of the gastrointestinal tract: review and update

“…7 More recently, an updated clinical prognostic model has been proposed specifically for EATL, the EPI score, which seems to perform better than the IPI and the PIT. 51 Pitfalls and pearls EATL can potentially be overlooked in ulcerated lesions obscured by inflammatory cells. There is a risk of misdiagnosing EATL as ALK-negative anaplastic large cell lymphoma in cases with CD30 expression (even more so when disease presents outside the GI tract).…”

The Risk of Malignancies in Celiac Disease—A Literature Review

“…The Prognostic Index for Peripheral T-cell Lymphoma (PIT), assessed by age, ECOG-PS, LDH levels, and bone marrow involvement [ 104 ], seems to have higher accuracy in predicting survival of EATL patients [ 83 ]. Indeed, among the four parameters considered for the prognostic stratification in the PIT model, only bone marrow involvement may be irrelevant for survival prediction of EATL patients, since it is present only in a few subjects [ 83 , 105 ]. More recently, a new clinical prognostic model integrating IPI variables with B-symptoms (fever or night sweats) was developed specifically for EATL patients (EATL Prognostic Index [EPI]) [ 105 ].…”

“…Indeed, among the four parameters considered for the prognostic stratification in the PIT model, only bone marrow involvement may be irrelevant for survival prediction of EATL patients, since it is present only in a few subjects [ 83 , 105 ]. More recently, a new clinical prognostic model integrating IPI variables with B-symptoms (fever or night sweats) was developed specifically for EATL patients (EATL Prognostic Index [EPI]) [ 105 ]. Three risk groups are identified by the EPI score: patients with B-symptoms (high-risk group: median overall survival [OS] 2 months); patients with an IPI score ≥2 and no B-symptoms (intermediate risk group: median OS of 7 months); patients with an IPI score of 0 or 1 and no B-symptoms (low-risk group: median OS 34 months) [ 105 ].…”

“…More recently, a new clinical prognostic model integrating IPI variables with B-symptoms (fever or night sweats) was developed specifically for EATL patients (EATL Prognostic Index [EPI]) [ 105 ]. Three risk groups are identified by the EPI score: patients with B-symptoms (high-risk group: median overall survival [OS] 2 months); patients with an IPI score ≥2 and no B-symptoms (intermediate risk group: median OS of 7 months); patients with an IPI score of 0 or 1 and no B-symptoms (low-risk group: median OS 34 months) [ 105 ]. The addition of B-symptoms as a parameter in EPI model resulted in improved recognition of the patients with an extremely poor prognosis needing more aggressive therapies; although very promising, it lacks an independent external validation process.…”

“…Prospective controlled or randomized clinical trials are difficult to perform because of the low incidence of this malignancy, the wide spectrum of its clinical presentation, its complex diagnosis, and malnutrition or poor performance status, which prevent the possibility of active therapies. Considering the difficulties involved in treatment, these patients have a very poor prognosis, with a 5-year survival rate lower than 20% [ 66 , 67 , 68 , 75 , 83 , 89 , 93 , 105 , 125 , 132 ]. A large retrospective study involving 37 patients with EATL revealed that the type of CeD (RCD-I vs. RCD-II), serum albumin levels, completion of at least one cycle of chemotherapy, and surprisingly surgical tumor resection were predictors of overall survival (OS) [ 75 ].…”