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Clinical Trial
. 2020 Feb;95(2):151-155.
doi: 10.1002/ajh.25674. Epub 2019 Nov 25.

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  1 , Steven M Horwitz  2 , Monica Civallero  3 , Monica Bellei  4 , Luigi Marcheselli  4 , Won Seog Kim  5 , Maria E Cabrera  6 , Ivan Dlouhy  7 , Arnon Nagler  8 , Ranjana H Advani  9 , Emanuela A Pesce  4 , Young-Hyeh Ko  10 , Silvia Montoto  11 , Carlos Chiattone  12 , Alison Moskowitz  2 , Michele Spina  13 , Marina Cesaretti  4 , Irene Biasoli  14 , Massimo Federico  15
Affiliations
Clinical Trial

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 et al. Am J Hematol. 2020 Feb.

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.

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Figures

FIGURE 1
FIGURE 1
Survival and progression free survival. Survival and PFS for HSTCL (A,D), Enteropathy associated T cell lymphoma (B,E), and PGDTCL (C,F)
See this image and copyright information in PMC

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