Oka Tomonori, Miyagaki Tomomitsu
Department of Dermatology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan.
Front Med (Lausanne). 2019 May 29;6:116. doi: 10.3389/fmed.2019.00116. eCollection 2019.
Mycosis fungoides (MF) and Sézary syndrome (SS) are the most common subtypes of cutaneous T-cell lymphoma. The majority of MF cases present with only patches and plaques and the lesions are usually limited to the skin. On the other hand, in some cases, patients show skin tumors or erythroderma followed by lymph node involvement and rarely visceral organ involvement. SS is a rare, aggressive cutaneous T-cell lymphoma marked by exfoliative erythroderma, lymphadenopathy, and leukemic blood involvement. Because patients with relapsed or refractory MF/SS display a poor prognosis and the current treatment options are characterized by high rates of relapse, there is unmet need for the efficient treatment. This review provides a discussion of the recent and future promising therapeutic approaches in the management of advanced MF/SS. These include mogamulizumab, brentuximab vedotin, alemtuzumab, immune checkpoint inhibitors, IPH4102 (anti-KIR3DL2 antibody), histone deacetylase inhibitors (vorinostat, romidepsin, panobinostat, belinostat, and resminostat), pralatrexate, forodesine, denileukin diftitox, duvelisib, lenalidomide, and everolimus.
蕈样肉芽肿(MF)和塞扎里综合征(SS)是皮肤T细胞淋巴瘤最常见的亚型。大多数MF病例仅表现为斑片和斑块,病变通常局限于皮肤。另一方面,在某些情况下,患者会出现皮肤肿瘤或红皮病,随后出现淋巴结受累,很少累及内脏器官。SS是一种罕见的侵袭性皮肤T细胞淋巴瘤,其特征为剥脱性红皮病、淋巴结病和白血病性血液受累。由于复发或难治性MF/SS患者预后较差,且目前的治疗选择具有高复发率的特点,因此对有效治疗存在未满足的需求。本综述讨论了晚期MF/SS治疗中近期和未来有前景的治疗方法。这些方法包括莫加莫珠单抗、本妥昔单抗、阿仑单抗、免疫检查点抑制剂、IPH4102(抗KIR3DL2抗体)、组蛋白去乙酰化酶抑制剂(伏立诺他、罗米地辛、帕比司他、贝利司他和雷斯司他)、普拉曲沙、福多司坦、地尼白介素-毒素融合蛋白、度维利西布、来那度胺和依维莫司。