Maria K. Angelopoulou*, John Asimakopoulos, Iliana Konstandinou, Theodoros P. Vassilakopoulos, Gerasimos Tsourouflis, Marina P Siakantaris and Kostas Konstantopoulos
Department of Hematology, Athens University Medical School, GreeceFulltext PDF
Peripheral T-cell Lymphomas (PTCL) arise from mature T-cells and they represent an extremely heterogeneous group. They are sub-classified into three major groups based on clinical presentation and localization, namely thenodal, extra nodal and leukemic PTCL. This review focuses on nodal PTCL which are the most frequently encountered entities. Nodal PTCL are divided into three basic categories: systemic anaplastic large-cell lymphoma (ALK+ sALCL and ALK- sALCL), Angioimmunoblastic T-Cell Lymphoma (AITL) and peripheral T-cell lymphoma not otherwise specified (PTCL-NOS).Nodal PTCL have a poor outcome with a 5-year Progression Free Survival (PFS) and Overall Survival (OS) of 25% to 38% and 30% to 45%, respectively. There is no standard treatment for these malignancies, so that the therapeutic strategy used in aggressive B-lymphomas has been adopted. Anthracycline-containing chemotherapy like CHOP is more often applied in the front line.The addition of etoposide to classic CHOP seems to favour patients under 60 years and especially those with ALK+ sALCL. Autologous Transplantation (ASCT) is used either as consolidation therapy following first complete remission or at relapse after salvage chemotherapy. Allogeneic transplantation (allo-SCT) is an option usually after ASCT failure. Newer agents are investigated mainly in the Relapsed/ Refractory (R/R) setting, while, the most activeones are being tested in front-line treatment, usually in combination with chemotherapy. These include nucleoside analogues (the main agent of this group is gemcitabine), antifolates such as plaratrexate and inhibitors of histone deacetylase likeromidepsin and belinostat. Among the newer agents, BrentuximabVedotin (BV)- an anti-CD30 antibody drug conjugate - has impressive activity in sALCLand has changed the dismal prognosis of R/R sALCL, especially ALK-. Lately, progress has been made in the understanding of the biology of PTCL. Therefore, new biological subsets have been identified which are characterized by activation of intracellular pathways offering new potential therapeutic targets. However, despite the plethora of new therapeutic agents, the natural history and prognosis of PTCL have not substantially improved yet. Hopefully, the understanding of the biological heterogeneity of these malignancies may enable a successful treatment approach. Multiple and overlapping pathways are possibly involved in the pathogenesis of these entities; clinical research should investigate the combination of chemotherapy with biological agents.
Angelopoulou MK, Asimakopoulos J, Konstandinou I, Vassilakopoulos TP, Tsourouflis G, Siakantaris MP, et al. Nodal Peripheral T-cell Lymphomas: Progress in the Treatment. Clin Oncol. 2018; 3: 1492.