Researchers evaluated treatment characteristics and outcomes of 3 rare subtypes of T-cell lymphoma, and their findings were published in the American Journal of Hematology.

The prospective study assessed treatments and clinical outcomes for patients with enteropathy-associated T-cell lymphoma (EATL), hepatosplenic T-cell lymphoma (HSTCL), and peripheral gamma delta T-cell lymphoma (PGDTCL). Patient cases were identified from the T Cell Project ( Identifier: NCT01142674) database, and therapies were guided by treating physicians.

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Among 1553 eligible patient cases, a total of 65 patients (4.2%) were diagnosed with EATL, 31 (2%) with HSTCL, and 19 (1.2%) with PGDTCL.

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Median study follow-up was 77 months for patients with PGDTCL, 64 months for patients with HSTCL, and 32 months for patients with EATL.

The 5-year overall survival (OS) rates were 30% with EATL, 40% with HSTCL, and 51% with PGDTCL. Median progression-free survival (PFS) was shortest for patients with EATL, at 7 months (95% CI, 4-10). For patients with HSTCL, median PFS was 11 months (95% CI, 8-14), and for patients with PGDTCL, median PFS was 14 months (95% CI, 6-21).

Treatment data were evaluable for 93 patients. All evaluable patients with PGDTCL and 97% of those with EATL received anthracycline-based therapies, compared with 60% of evaluable patients with HSCTL. Complete response rates to initial chemotherapy regimens were 30% for EATL, 40% for HSCTL, and 25% for PGDTCL. Autologous stem cell transplantation was used during first remission in a minority of patients with each of the 3 subtypes of T-cell lymphoma.

The study investigators highlighted the value of registries in studies of rare diseases. In the case of this study, the investigators noted a lack of standardized treatment practices for the examined T-cell lymphoma subtypes, and they recommended that patients with rare T-cell lymphoma subtypes be included in studies of novel agents.


  1. Foss FM, Horwitz SM, Civallero M, et al. Incidence and outcomes of rare T cell lymphomas from the T Cell Project: hepatosplenic, enteropathy associated and peripheral gamma delta T cell lymphomas [published online November 10, 2019]. Am J Hematol. doi:10.1002/ajh.25674