Abstract
Peripheral T-cell lymphomas (PTCLs) are a rare and heterogenous subset of non-Hodgkin lymphoma characterized by an aggressive clinical course. Historically, the treatment of PTCLs have been analogous to that of aggressive B-cell lymphomas; however, it has been well-established that overall responses and complete remission rates are far inferior using near-identical chemotherapy strategies. Recently, there has been a plethora of newer agents designed to target distinguishing cellular and molecular features of specific PTCL subtypes. These agents have been proven to yield superior anti-lymphoma responses and, in some cases, overall survival in the relapsed, refractory, and frontline treatment setting. In this review, we will summarize and highlight the most influential clinical trials leading to the Food and Drug Administration (FDA) approval of several novel therapeutic agents against PTCL, with an emphasis on emerging studies and strategies to expand their potential use in the frontline treatment setting.
Highlights
Peripheral T-cell lymphomas (PTCL) represent a spectrum of hematological diseases that account for 5–10% of non-Hodgkin Lymphoma (NHL) and 15–20% of aggressive lymphomas in Western countries [1]
A single-institution, retrospective analysis demonstrated for 117 patients with PTCL-unspecified (PTCL-U, the most common histological subtype prior to the World Health Organization (WHO) designation of PTCL-NOS) receiving CHOP-based chemotherapy, an overall response rate (ORR) of 84% and a complete response (CR) in 64% of patients was observed; the 5-year progression-free survival (PFS) was only 29% and 5-year overall survival (OS) was 35% [9]
83 patients (PTCL-NOS: n = 32 and angioimmunoblastic T-cell lymphoma (AITL): n = 27) underwent induction, with 66% of patients continuing to autologous stem cell transplantation (ASCT); the majority of remaining patients did not continue with ASCT due to progressive disease
Summary
Peripheral T-cell lymphomas (PTCL) represent a spectrum of hematological diseases that account for 5–10% of non-Hodgkin Lymphoma (NHL) and 15–20% of aggressive lymphomas in Western countries [1]. A retrospective analysis of patients with PTCL treated in studies of the German HighGrade Non-Hodgkin Lymphoma Study Group revealed that those younger than 60 years of age and with LDH levels less than the upper limit of normal per laboratory standard at the time of diagnosis had a 3-year event-free survival benefit when treated with CHOEP as opposed to CHOP in the frontline setting (75.4% vs 51.0%, respectively) [27] This led to a multicenter, phase II study to evaluate the addition of etoposide to BV + CHP (CHEPBV), followed by BV consolidation in patients with newly diagnosed CD30-expressing PTCL. Belinostat represents a potentially universal adjunct to standard CHOP chemotherapy in the frontline setting in non-ALCL PTCL and may potentially have less hematological toxicities than Ro-CHOP, pending assessment in a randomized trial
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