Incidence and outcomes of rare T cell lymphomas from the T Cell Project: hepatosplenic, enteropathy associated and peripheral gamma delta T cell lymphomas

Francine M. Foss, Steven M. Horwitz, Monica Civallero, Monica Bellei, Luigi Marcheselli, Won Seog Kim, Maria E. Cabrera, Ivan Dlouhy, Arnon Nagler, Ranjana H. Advani, Emanuela A. Pesce, Young Hyeh Ko, Silvia Montoto, Carlos Chiattone, Alison Moskowitz, Michele Spina, Marina Cesaretti, Irene Biasoli, Massimo Federico

Research output: Contribution to journalArticlepeer-review

Abstract

The T Cell Project was the largest prospective trial to explore the incidence, treatment patterns, and outcomes for T cell lymphomas. The rare subtypes of T cell lymphomas, including hepatosplenic T cell lymphoma (HSTCL), enteropathy associated T cell lymphoma (EATL), and peripheral gamma delta T cell lymphomas (PGDTCLs) are poorly represented in most studies and there is little data regarding treatment patterns. We report results from 115 patients with hepatosplenic (n = 31), enteropathy associated (n = 65), and PGDTCLs (n = 19). While anthracycline regimens were most commonly used as first line therapy, response rates ranged from 20%-40% and were suboptimal for all groups. Autologous stem cell transplantation was performed as a consolidation in first remission in a small number of patients (33% of HSTCL, 7% of EATL, and 12% of PGDTCL), and four patients with HSTCL underwent allogeneic stem cell transplantation in first remission. The progression free survival at 3 years ranged from 28%-40% for these rare subtypes, and the overall survival at 3 years was most favorable for PGDTCL (70%). These data highlight the need for novel treatment approaches for rare subtypes of T cell lymphomas and for their inclusion in clinical trials.

Original languageEnglish
Pages (from-to)151-155
Number of pages5
JournalAmerican Journal of Hematology
Volume95
Issue number2
DOIs
Publication statusPublished - Feb 1 2020

ASJC Scopus subject areas

  • Hematology

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