Enteropathy-associated T-cell lymphoma (types I and II) is a rare, mature T-cell lymphoma that may present anywhere within the gastrointestinal tract but most commonly the jejunum or ileum. Retrospective series of patients with EATL report a very poor prognosis with median survival in the range of 7 months. the recent ASCO Educational Book says the following: “Although in EATL, as in other subtypes of PTCL, CHOP often leads to suboptimal results, there is evidence, albeit inconclusive
evidence, that more aggressive approaches do result in better long-term outcomes. As such, we tend to follow the largest phase 2,
Nordic Trial, as a treatment paradigm and use CHOEP followed by ASCT as our most commonly used approach. We are intrigued by
the results of IVE/MTX-ASCT and at least consider it in very young and fit patients.
Sieniawski M et al, Evaluation of enteropathy-associated T-cell lymphoma comparing standard therapies with a novel regimen including autologous stem cell transplantation. Blood. 2010 May 6;115(18):3664-70.
Neha Mehta–Shah et al, Zebras and hen’s teeth: recognition and management of rare T and NK lymphomas. ASH Educational Book 2015.
Antonio Di Sabatino et al, How I treat enteropathy-associated T-cell lymphoma. Blood. 2012;119(11):
2458-2468)