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