Abstract
Owing to the rarity of peripheral T-cell lymphoma (PTCL) and the heterogeneity of subtypes, there are no compelling data to guide the therapeutic approaches for such patients. Over the years, there have been remarkable advances in molecular subtyping and treatment of PTCL, although there are still many areas to be explored. In this review, we summarize recent updates on the evolution of understanding and treatment for PTCL.
Highlights
T/natural killer (T/NK) cell lymphoma represents a heterogeneous group of malignant lymphoproliferative diseases that arise from T-lymphocytes and NK cells
We provide an overview of the latest advances in the management of peripheral T-cell lymphoma (PTCL) focusing on trials that have been carried out with novel agents
Brief review of the 2016 World Health Organization revision of nodal/extranodal peripheral T-cell lymphomas Beginning with nodal PTCLs, up to 60–100% of angioimmunoblastic T-cell lymphoma (AITL) and up to 40% of PTCL not otherwise specified (PTCL-NOS) demonstrate surface markers of follicular helper T (TFH) cells[4,5,6,7] and have common genetic features, such as RHOA, TET2, DNMT3A, and IDH8–11
Summary
T/natural killer (T/NK) cell lymphoma represents a heterogeneous group of malignant lymphoproliferative diseases that arise from T-lymphocytes and NK cells. Owing to frequent relapse after remission, the three-year OS and PFS rates were 47% and 35%, respectively In another trial by the French group, the combination of bortezomib plus intensified CHOP-like regimen (ACVBP) was studied in 57 patients with newly diagnosed PTCL115. In a phase II study of 30 patients with untreated PTCL who received everolimus plus CHOP123, CR was observed in 17 (57%) and PR was observed in 10 (33%) Despite these favorable tumor responses, frequent relapses were noted as the two-year PFS rate was 33%. A phase III LUMIERE (Alisertib or Investigator’s Choice in Patients With Relapsed/Refractory Peripheral T-Cell Lymphoma) trial was performed in patients with rrPTCL to compare the efficacy of alisertib versus the investigator’s choice, including pralatrexate, romidepsin, or gemcitabine[130]. Out of five evaluable patients, three CRs and one PR were observed
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