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Crit Rev Oncol Hematol. 2016 Mar;99:228-40. doi: 10.1016/j.critrevonc.2015.12.018. Epub 2016 Jan 2.

Panoptic clinical review of the current and future treatment of relapsed/refractory T-cell lymphomas: Cutaneous T-cell lymphomas.

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Institute of Hematology 'L. e A. Seràgnoli', Policlinico Sant'Orsola-Malpighi, University of Bologna, Via Massarenti 9, 40138 Bologna, Italy. Electronic address:
Global Outcomes and Epidemiology Research (GOER), Millennium Pharmaceuticals Inc., 40 Lansdowne Street, Cambridge, MA 02139, USA(1).
Oncology Clinical Research, Millennium Pharmaceuticals Inc., 35 Lansdowne Street, Cambridge, MA 02139, USA(1).
Department of Biostatistics, Millennium Pharmaceuticals Inc., 40 Lansdowne Street, Cambridge, MA 02139, USA(1).
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.


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.


Bexarotene; Brentuximab vedotin; Cutaneous T-cell lymphoma; Mycosis fungoides; Romidepsin; Sézary syndrome; Vorinostat

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