title: Panoptic clinical review of the current and future treatment of relapsed/refractory T-cell lymphomas: Cutaneous T-cell lymphomas.
abstract: Primary cutaneous T-cell lymphomas (CTCLs), such as mycosis fungoides and Sézary syndrome, are a rare group of non-Hodgkin lymphomas, usually treated using a multimodal approach. Unfortunately, many patients go on to develop relapsed/refractory disease. Systemic treatment for relapsed/refractory CTCL has historically relied on chemotherapies and interferons, and while active, responses are often short-lived. Three drugs are now approved in the US to treat relapsed/refractory CTCL including the oral retinoid, bexarotene, and histone deacetylase inhibitors, romidepsin and vorinostat. Although response rates are typically <35%, romidepsin and vorinostat can induce some durable responses in heavily pretreated patients and alleviate bothersome symptoms, such as pruritus. New studies indicate that the anti-CD30 antibody-drug conjugate brentuximab vedotin, anti-CCR4 antibody mogamulizumab, and fusion protein immunotoxin A-dmDT390-bisFv(UCHT1) may be particularly active in this setting. In this paper, we present an exhaustive review of the clinical data on current and possible future drug treatment options for relapsed/refractory CTCL.
authors.affiliation: Institute of Hematology 'L. e A. Seràgnoli', Policlinico Sant'Orsola-Malpighi, 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
authors.affiliation: Global Outcomes and Epidemiology Research (GOER), Millennium Pharmaceuticals Inc., 40 Lansdowne Street, Cambridge, MA 02139, USA(1).
authors.forename: Vijayveer
authors.initials: V
authors.lastname: Bonthapally
authors.affiliation: Oncology Clinical Research, Millennium Pharmaceuticals Inc., 35 Lansdowne Street, Cambridge, MA 02139, USA(1).
authors.forename: Dirk
authors.initials: D
authors.lastname: Huebner
authors.affiliation: Oncology Clinical Research, Millennium Pharmaceuticals Inc., 35 Lansdowne Street, Cambridge, MA 02139, USA(1).
authors.forename: Richard
authors.initials: R
authors.lastname: Lutes
authors.affiliation: Department of Biostatistics, Millennium Pharmaceuticals Inc., 40 Lansdowne Street, Cambridge, MA 02139, USA(1).
authors.forename: Andy
authors.initials: A
authors.lastname: Chi
authors.affiliation: Department of Experimental, Diagnostic, and Specialty Medicine, Bologna University School of Medicine, Via Massarenti 8, 40138 Bologna, Italy; Unit of Hematopathology, European Institute of Oncology, Via Ripamonti 435, Milan 20141, Italy.
authors.forename: Stefano
authors.initials: S
authors.lastname: Pileri
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D019008:Drug Resistance, Neoplasm / Q000187:drug effects*; D006801:Humans; D016410:Lymphoma, T-Cell, Cutaneous / Q000188:drug therapy*; D009364:Neoplasm Recurrence, Local / Q000188:drug therapy*; D016879:Salvage Therapy; D012878:Skin Neoplasms / Q000188:drug therapy*
keywords: Bexarotene; Brentuximab vedotin; Cutaneous T-cell lymphoma; Mycosis fungoides; Romidepsin; Sézary syndrome; Vorinostat