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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. © 2019 Wiley Periodicals, Inc.


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. Incidence and outcomes of rare T cell lymphomas from the T Cell Project: hepatosplenic, enteropathy associated and peripheral gamma delta T cell lymphomas. American journal of hematology. 2020 Feb;95(2):151-155

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PMID: 31709579

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