title: Therapeutic options in relapsed or refractory peripheral T-cell lymphoma.
abstract: Peripheral T-cell lymphoma (PTCL) represents a relatively rare group of heterogeneous non-Hodgkin lymphomas with a very poor prognosis. Current therapies, based on historical regimens for aggressive B-cell lymphomas, have resulted in insufficient patient outcomes. The majority of patients relapse rapidly, and current 5-year overall survival rates are only 10-30%. It is evident that new approaches to treat patients with PTCL are required. In recent years, prospective studies in PTCL have been initiated, mainly in patients with relapsed/refractory disease. In some of these, selected histologic subtypes have been evaluated in detail. As a consequence, numerous new therapies have been developed and shown activity in PTCL, including: agents targeting the immune system (e.g. brentuximab vedotin, alemtuzumab, lenalidomide); histone deacetylase inhibitors (romidepsin, belinostat); antifolates (pralatrexate); fusion proteins (denileukin diftitox); nucleoside analogs (pentostatin, gemcitabine); and other agents (e.g. alisertib, plitidepsin, bendamustine, bortezomib). A variety of interesting novel combinations is also emerging. It is hoped that these innovative approaches, coupled with a greater understanding of the clinicopathologic features, pathogenesis, molecular biology, and natural history of PTCL will advance the field and improve outcomes in this challenging group of diseases. This review summarizes the currently available clinical evidence on the various approaches to treating relapsed/refractory PTCL, including the role of stem cell transplantation, with an emphasis on potential new drug therapies.
authors.affiliation: University of Lyon 1, 17 Rue de France, 69627 Lyon, France. Electronic address: bertrand.coiffier@univ-lyon1.fr.
authors.forename: Bertrand
authors.initials: B
authors.lastname: Coiffier
authors.affiliation: Dipartimento di Medicina di Laboratorio, Clinica e di Sanità Pubblica, Università degli studi di Modena e Reggio Emilia, Policlinico, Via del Pozzo, 71, 41124 Modena, Italy. Electronic address: massimo.federico@unimore.it.
authors.forename: Massimo
authors.initials: M
authors.lastname: Federico
authors.affiliation: Instituto Biosanitario de Salamanca, Paseo de San Vicente 58-182, 37007 Salamanca, Spain. Electronic address: cabarri@usal.es.
authors.forename: Dolores
authors.initials: D
authors.lastname: Caballero
authors.affiliation: Department of Haemato-Oncology, Royal Marsden Hospital, Downs Road, SM2 5PT Sutton, UK. Electronic address: claire.dearden@rmh.nhs.uk.
authors.forename: Claire
authors.initials: C
authors.lastname: Dearden
authors.affiliation: Department of Hematology, University Hospital of Lille, F-59037 Lille, France. Electronic address: franck.morschhauser@chru-lille.fr.
authors.forename: Franck
authors.initials: F
authors.lastname: Morschhauser
authors.affiliation: Medical University of Vienna, Department of Medicine I, Division of Hematology and Hemostaseology, Comprehensive Cancer Center, Waehringer Guertel 18-20, 1090 Vienna, Austria. Electronic address: ulrich.jaeger@meduniwien.ac.at.
authors.forename: Ulrich
authors.initials: U
authors.lastname: Jäger
authors.affiliation: UniversitätsKrebszentrum (G-CCC), Georg August University, 37099 Göttingen, Germany. Electronic address: lorenz.truemper@med.uni-goettingen.de.
authors.forename: Lorenz
authors.initials: L
authors.lastname: Trümper
authors.affiliation: Oncology Institute of Southern Switzerland (IOSI), Ospedale San Giovanni, 6500 Bellinzona, Switzerland. Electronic address: emanuele.zucca@eoc.ch.
authors.forename: Emanuele
authors.initials: E
authors.lastname: Zucca
authors.affiliation: Instituto Português de Oncologia de Lisboa de Francisco Gentil, R. Prof. Lima Basto, 1099-023 Lisbon, Portugal. Electronic address: mgsilva@ipolisboa.min-saude.pt.
authors.forename: Maria
authors.initials: M
authors.lastname: Gomes da Silva
authors.affiliation: St George's University of London, Cranmer Terrace, London SW17 0RE, UK. Electronic address: r.pettengell@sgul.ac.uk.
authors.forename: Ruth
authors.initials: R
authors.lastname: Pettengell
authors.affiliation: Klinik für Onkologie und Hämatologie am Krankenhaus Nordwest GmbH, Steinbacher Hohl 2-26, D-60488 Frankfurt, Germany. Electronic address: weidmann.eckhart@khnw.de.
authors.forename: Eckhart
authors.initials: E
authors.lastname: Weidmann
authors.affiliation: Department Hematology, Aarhus University Hospital, Tage Hansens Gade 2, DK-8000 Aarhus C, Denmark. Electronic address: frandamo@rm.dk.
authors.forename: Francesco
authors.initials: F
authors.lastname: d'Amore
authors.affiliation: Department of Hematology, Centre Henri-Becquerel, UMR918, Université de Rouen, Rue d'Amiens, 76038 Rouen Cedex 1, France. Electronic address: herve.tilly@rouen.fnclcc.fr.
authors.forename: Hervé
authors.initials: H
authors.lastname: Tilly
authors.affiliation: Institute of Hematology "Seràgnoli", University of Bologna, Via Massarenti 9, 40138 Bologna, Italy. Electronic address: pierluigi.zinzani@unibo.it.
authors.forename: Pier Luigi
authors.initials: PL
authors.lastname: Zinzani
mesh_terms: D000074323:Alemtuzumab; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use; D000970:Antineoplastic Agents / Q000494:pharmacology / Q000627:therapeutic use*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000494:pharmacology / Q000627:therapeutic use; D000079963:Brentuximab Vedotin; D003520:Cyclophosphamide / Q000627:therapeutic use; D047630:Depsipeptides / Q000627:therapeutic use; D004167:Diphtheria Toxin / Q000627:therapeutic use; D004317:Doxorubicin / Q000627:therapeutic use; D005493:Folic Acid Antagonists / Q000494:pharmacology / Q000627:therapeutic use; D056572:Histone Deacetylase Inhibitors / Q000627:therapeutic use; D006801:Humans; D018796:Immunoconjugates / Q000627:therapeutic use; D007376:Interleukin-2 / Q000627:therapeutic use; D000077269:Lenalidomide; D016411:Lymphoma, T-Cell, Peripheral / Q000188:drug therapy* / Q000473:pathology* / Q000628:therapy; D009364:Neoplasm Recurrence, Local / Q000188:drug therapy; D010456:Peptides, Cyclic; D011239:Prednisolone / Q000627:therapeutic use; D011993:Recombinant Fusion Proteins / Q000627:therapeutic use; D033581:Stem Cell Transplantation; D013792:Thalidomide / Q000031:analogs & derivatives / Q000627:therapeutic use; D059003:Topoisomerase Inhibitors / Q000494:pharmacology / Q000627:therapeutic use; D014750:Vincristine / Q000627:therapeutic use
chemical_list: D061067:Antibodies, Monoclonal, Humanized; D000970:Antineoplastic Agents; D047630:Depsipeptides; D004167:Diphtheria Toxin; D005493:Folic Acid Antagonists; D056572:Histone Deacetylase Inhibitors; D018796:Immunoconjugates; D007376:Interleukin-2; D010456:Peptides, Cyclic; D011993:Recombinant Fusion Proteins; D059003:Topoisomerase Inhibitors; C078456:denileukin diftitox; D000074323:Alemtuzumab; D013792:Thalidomide; D014750:Vincristine; D000079963:Brentuximab Vedotin; D004317:Doxorubicin; D003520:Cyclophosphamide; D011239:Prednisolone; C087123:romidepsin; D000077269:Lenalidomide; C098980:plitidepsin
keywords: Antifolate; Fusion protein; Histone deacetylase inhibitor; Immunomodulatory agent; Nucleoside analog; Peripheral T-cell lymphoma