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 Foss, F. M., Horwitz, S. M., Civallero, M., Bellei, M., Marcheselli, L., Kim, W. S., Cabrera, M. E., Dlouhy, I., Nagler, A., Advani, R. H., Pesce, E. A., Ko, Y., Montoto, S., Chiattone, C., Moskowitz, A., Spina, M., Cesaretti, M., Biasoli, I., Federico, M. 2019


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 PGDTCLs (PGDTCL) 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 4 patients with HSTCL underwent allogeneic stem cell transplantation in first remission. The progression free survival at 3years ranged from 28-40% for these rare subtypes, and the overall survival at 3years 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. This article is protected by copyright. All rights reserved.

View details for DOI 10.1002/ajh.25674

View details for PubMedID 31709579