Prince H Miles, Querfeld Christiane
Epworth Healthcare and Sir Peter MacCallum Department of Oncology, University of Melbourne, 140 Clarendon Street, East Melbourne, Victoria, Australia.
City of Hope National Medical Center, Beckman Research Institute, 1500 E. Duarte Road, Duarte, CA 91010, USA.
Best Pract Res Clin Haematol. 2018 Sep;31(3):322-335. doi: 10.1016/j.beha.2018.07.007. Epub 2018 Jul 18.
Novel systemic therapies are generally prescribed to patients with advanced-stage disease or those with early-stage disease refractory to skin-directed therapies. In general, systemic chemotherapy should be reserved for patients who fail to respond to biological agents. Such biological agents include interferon alfa, bexarotene, histone deacetylase inhibitors (vorinostat, romidepsin), brentuximab vedotin and mogamulizumab. Extracorporeal photopheresis is particularly effective for patients with Sézary Syndrome. Allogeneic transplantation is becoming increasing used for younger patients. Novel agents in advanced development include the monoclonal antibody IPH4102,duvelisib,and the new modified formulation of denileukin diftitox. The choice of agents for patients is typically a balance of patient factors (age, co-morbidities, geographic location), relative efficacy and toxicity.
新型全身治疗通常用于晚期疾病患者或对皮肤定向治疗难治的早期疾病患者。一般来说，全身化疗应保留给对生物制剂无反应的患者。此类生物制剂包括干扰素α、贝沙罗汀、组蛋白脱乙酰酶抑制剂（伏立诺他、罗米地辛）、本妥昔单抗和莫加莫单抗。体外光化学疗法对 Sézary 综合征患者特别有效。异基因移植越来越多地用于年轻患者。处于晚期研发阶段的新型药物包括单克隆抗体 IPH4102、度维利西布以及地尼白介素 - 妥西毒素的新改良制剂。为患者选择药物通常是患者因素（年龄、合并症、地理位置）、相对疗效和毒性之间的平衡。
