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

NCCN Guidelines Insights: T-Cell Lymphomas, Version 1.2021

“…Hepatosplenic T-cell lymphoma (HSTCL) is a rare lymphoproliferative disorder associated with an aggressive clinical course and a worse prognosis. 1,2 HSTCL accounts for #2% of all cases of T-cell lymphoma diagnosed worldwide, and in up to 20% of cases develops in the setting of chronic immune suppression or immune dysregulation particularly inflammatory bowel disease (IBD), hematologic malignancies, and previous solid organ transplant. 3,4 The concomitant use of tumor necrosis factor-a (TNF-a) inhibitors and thiopurine-based immunomodulators has been identified as a risk factor for developing HSTCL among patients with IBD.…”

Hematemesis as an Initial Presentation of Enteropathy-Associated T-Cell Lymphoma

“…EATL is peripheral or extranodal T-cell lymphoma arising in the small intestine and is commonly associated with CD [ 1 ]. It accounts for 4.2% of all peripheral T-cell lymphomas, which is more common in Europeans due to the increased incidence of celiac disease [ 2 ]. In the US, the incidence rate was 0.111 per 1,000,000, with a higher incidence in Asians/Pacific Islanders [ 3 , 4 ].…”

Effect of chidamide on treating hepatosplenic T-cell lymphoma: A case report

“…The first-line chemotherapy regimen of chemo-with-anthracycline regimen (CHOP, CVP, or Hyper CVAD/MA) is the top priority in majority of the patients to start with as the induction option. However, poor prognosis with much lower CR response rate (20%-40%)[ 11 ] as compared to that of nodal T-cell lymphomas and short time of relapse (8-16 mo) has long been the major concern.…”

“…Additionally, three patients underwent SCT in the salvage setting. Therefore, these patients with HSTCL still had a shortened median OS (13 mo) and a shortened median progression-free survival (11 mo)[ 11 ].…”