title: Peripheral T-cell lymphoma, NOS, and anaplastic large cell lymphoma.
abstract: Peripheral T-cell lymphomas (PTCL), with the exception of anaplastic lymphoma kinase (ALK)-positive anaplastic large cell lymphoma (ALCL), have a very poor prognosis. Although current first line chemotherapy continues to be a CHOP-like (cyclophosphamide, doxorubicin, vincristine, prednisone) regimen there is now data suggesting that the addition of etoposide in younger patients improves outcomes. Even for those patients who do have a response to therapy, the risk of relapse remains quite high. Although autologous transplant in first remission is often used, its role as consolidation therapy in first remission remains unclear and may preferentially benefit low-risk patients. In the relapsed setting, major advances have occurred with Food and Drug Administration (FDA) approval of 4 new agents (pralatrexate, romidepsin, belinostat, brentuximab vedotin) for relapsed/refractory PTCL since 2009. These 4 drugs represent the first agents ever approved specifically for this indication. Unfortunately, with the exception of ALCL for which brentuximab vedotin will likely substantially change our approach to treatment, there are still many patients for whom available drugs will not be effective, and it is for these patients that further advances are urgently needed.
authors.affiliation: Duke University Medical Center, Durham, NC.
authors.forename: Anne W
authors.initials: AW
authors.lastname: Beaven
authors.affiliation: Duke University Medical Center, Durham, NC.
authors.forename: Louis F
authors.initials: LF
authors.lastname: Diehl
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use; D000971:Antineoplastic Combined Chemotherapy Protocols; D003520:Cyclophosphamide; D018572:Disease-Free Survival; D004317:Doxorubicin; D017277:Drug Approval; D005047:Etoposide / Q000627:therapeutic use; D005060:Europe; D006801:Humans; D007391:International Cooperation; D017728:Lymphoma, Large-Cell, Anaplastic / Q000188:drug therapy*; D016411:Lymphoma, T-Cell, Peripheral / Q000188:drug therapy*; D008495:Medical Oncology / Q000379:methods; D009364:Neoplasm Recurrence, Local; D009842:Oligopeptides / Q000627:therapeutic use; D011239:Prednisolone; D011379:Prognosis; D012008:Recurrence; D012074:Remission Induction; D012306:Risk; D016896:Treatment Outcome; D014481:United States; D014486:United States Food and Drug Administration; D014750:Vincristine
chemical_list: D000970:Antineoplastic Agents; D009842:Oligopeptides; D014750:Vincristine; D005047:Etoposide; D004317:Doxorubicin; D003520:Cyclophosphamide; D011239:Prednisolone; C495575:monomethyl auristatin E