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Incidence and outcomes of rare T cell lymphomas from the T Cell Project: hepatosplenic, enteropathy associated and peripheral gamma delta T cell lymphomas
Author(s) -
Foss Francine M.,
Horwitz Steven M.,
Civallero Monica,
Bellei Monica,
Marcheselli Luigi,
Kim Won Seog,
Cabrera Maria E.,
Dlouhy Ivan,
Nagler Ar,
Advani Ranjana H.,
Pesce Emanuela A.,
Ko YoungHyeh,
Montoto Silvia,
Chiattone Carlos,
Moskowitz Alison,
Spina Michele,
Cesaretti Marina,
Biasoli Irene,
Federico Massimo
Publication year - 2020
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.25674
Subject(s) - medicine , lymphoma , transplantation , peripheral t cell lymphoma , t cell lymphoma , autologous stem cell transplantation , gastroenterology , t cell , oncology , enteropathy , immunology , immune system , disease
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.