Incidence and outcomes of rare T cell lymphomas from the T Cell Project: hepatosplenic, enteropathy associated and peripheral gamma delta T cell lymphomas. Issue 2 (25th November 2019)

• Record Type: Journal Article
• Authors: Foss, Francine M.; Horwitz, Steven M.; Civallero, Monica; Bellei, Monica; Marcheselli, Luigi; Kim, Won Seog; Cabrera, Maria E.; Dlouhy, Ivan; Nagler, Arnon; Advani, Ranjana H.; Pesce, Emanuela A.; Ko, Young‐Hyeh; Montoto, Silvia; Chiattone, Carlos; Moskowitz, Alison; Spina, Michele; Cesaretti, Marina; Biasoli, Irene; Federico, Massimo
• Is Part Of: American journal of hematology. Volume 95:Issue 2(2020:Feb.)
• Page Start: 151
• Page End: 155
• Publication Date: 2019-11-25
• Subjects: Hematology -- Periodicals; 616.15
• DOI: 10.1002/ajh.25674
• Languages: English
• ISSNs: 0361-8609

Abstract

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.