Controversies in the Front-Line Treatment of Systemic Peripheral T Cell Lymphomas

Abstract

Systemic peripheral T cell lymphomas (PTCL) are a rare and clinically and biologically heterogeneous group of disorders with scarce and generally low-quality evidence guiding their management. In this manuscript, we tackle the current controversies in the front-line treatment of systemic PTCL including (1) whether CNS prophylaxis should be administered; (2) whether CHOEP should be preferred over CHOP; (3) what role brentuximab vedotin should have; (4) whether stem cell transplant (SCT) consolidation should be used and whether autologous or allogeneic; (5) how should molecular subtypes (including DUSP22 or TP63-rearranged ALCL or GATA3 or TBX21 PTCL, NOS) impact therapeutic decisions; and (6) whether there is a role for targeted agents beyond brentuximab vedotin.

Complete hematologic response in a patient with multiple pretreated angioimmunoblastic T‐cell lymphoma after belinostat therapy followed by allogeneic stem cell transplantation: A case report

“… 1 Typically diagnosed at advanced stage, PTCLs exhibit poor treatment outcomes, with a 5‐year survival rate of 30%–40%. 2 According to the World Health Organization (WHO) classification, mature T‐cell and NK‐cell neoplasms are classified based on a combination of morphologic, immunophenotypic, genetic, and clinical features. 3 The most prevalent PTCL subtypes include nodal follicular T helper cell lymphomas (nTFHL) whose prototype is nTFHL of the angioimmunoblastic‐type (nTFHL‐AI), PTCL not otherwise specified (PTCL‐NOS), and anaplastic large cell lymphoma (ALCL).…”

“…Peripheral T‐cell lymphomas (PTCLs) represent a heterogenous group of highly aggressive lymphoid malignancies, which are derived from mature T‐cells or natural killer (NK)/T‐cells and constitute approximately 15%–20% of all non‐Hodgkin's lymphomas (NHL) in Western populations 1 . Typically diagnosed at advanced stage, PTCLs exhibit poor treatment outcomes, with a 5‐year survival rate of 30%–40% 2 . According to the World Health Organization (WHO) classification, mature T‐cell and NK‐cell neoplasms are classified based on a combination of morphologic, immunophenotypic, genetic, and clinical features 3 .…”