Zinzani Pier Luigi, Bonthapally Vijayveer, Huebner Dirk, Lutes Richard, Chi Andy, Pileri Stefano
Institute of Hematology 'L. e A. Seràgnoli', Policlinico Sant'Orsola-Malpighi, University of Bologna, Via Massarenti 9, 40138 Bologna, Italy.
Global Outcomes and Epidemiology Research (GOER), Millennium Pharmaceuticals Inc., 40 Lansdowne Street, Cambridge, MA 02139, USA(1).
Crit Rev Oncol Hematol. 2016 Mar;99:228-40. doi: 10.1016/j.critrevonc.2015.12.018. Epub 2016 Jan 2.
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.
原发性皮肤T细胞淋巴瘤（CTCL），如蕈样肉芽肿和 Sézary 综合征，是一组罕见的非霍奇金淋巴瘤，通常采用多模式方法进行治疗。不幸的是，许多患者随后会发展为复发/难治性疾病。复发/难治性CTCL的全身治疗历来依赖于化疗和干扰素，虽然有疗效，但反应往往是短暂的。目前在美国有三种药物被批准用于治疗复发/难治性CTCL，包括口服维甲酸贝沙罗汀以及组蛋白去乙酰化酶抑制剂罗米地辛和伏立诺他。尽管缓解率通常<35%，但罗米地辛和伏立诺他可在经过大量预处理的患者中诱导出一些持久反应，并缓解瘙痒等令人烦恼的症状。新研究表明，抗CD30抗体药物偶联物本妥昔单抗、抗CCR4抗体莫加莫拉单抗和融合蛋白免疫毒素A-dmDT390-bisFv(UCHT1)在这种情况下可能特别有效。在本文中，我们对复发/难治性CTCL当前及未来可能的药物治疗选择的临床数据进行了详尽综述。
