Incidence and outcomes of rare T cell lymphomas from the T Cell Project: Hepatosplenic, enteropathy associated and peripheral gamma delta T cell lymphomas Journal Article
| Authors: | 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. H.; Montoto, S.; Chiattone, C.; Moskowitz, A.; Spina, M.; Cesaretti, M.; Biasoli, I.; Federico, M. |
| Article Title: | Incidence and outcomes of rare T cell lymphomas from the T Cell Project: Hepatosplenic, enteropathy associated and peripheral gamma delta T cell lymphomas |
| 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. © 2019 Wiley Periodicals, Inc. |
| Keywords: | adult; cancer chemotherapy; cancer survival; aged; major clinical study; overall survival; allogeneic stem cell transplantation; drug efficacy; treatment planning; outcome assessment; cancer incidence; autologous stem cell transplantation; survival time; cancer regression; peripheral t cell lymphoma; drug response; clinical effectiveness; anthracycline; rare disease; hepatosplenic t cell lymphoma; enteropathy associated t cell lymphoma; human; male; female; priority journal; article |
| Journal Title: | American Journal of Hematology |
| Volume: | 95 |
| Issue: | 2 |
| ISSN: | 0361-8609 |
| Publisher: | John Wiley & Sons, Inc. |
| Date Published: | 2020-02-01 |
| Start Page: | 151 |
| End Page: | 155 |
| Language: | English |
| DOI: | 10.1002/ajh.25674 |
| PUBMED: | 31709579 |
| PROVIDER: | scopus |
| PMCID: | PMC8025136 |
| DOI/URL: | |
| Notes: | Article -- Source: Scopus |
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