Khan Niloufer, Ozkaya Neval, Moskowitz Alison, Dogan Ahmet, Horwitz Steven
Lymphoma Service, Memorial Sloan Kettering Cancer Center, 1275 York Avenue, New York, NY 10065, USA.
Laboratory of Pathology, National Cancer Institute, Bethesda, MD, USA.
Best Pract Res Clin Haematol. 2018 Sep;31(3):306-314. doi: 10.1016/j.beha.2018.07.010. Epub 2018 Jul 17.
Peripheral T cell lymphoma (PTCL) is a rare subtype of non-Hodgkin lymphoma. PTCLs are heterogeneous in terms of biology, but generally have more aggressive features and poorer outcomes than aggressive B-cell lymphomas when treated with combination chemotherapy. While the best long-term results are still seen with intensive chemotherapeutic approaches, significant progress has been made with molecular profiling identifying genetic drivers of PTCL that could serve as therapeutic targets. Tailoring therapy to different subtypes of PTCL may lead to more individualized approaches with the hope of improved outcomes. In this paper, we review current therapies for treatment of PTCL, newly identified molecular markers, and the role of emerging therapy and novel combinations of existing agents.
外周T细胞淋巴瘤（PTCL）是一种罕见的非霍奇金淋巴瘤亚型。PTCL在生物学特性上具有异质性，但与侵袭性B细胞淋巴瘤相比，采用联合化疗治疗时通常具有更侵袭性的特征且预后较差。虽然强化化疗方法仍能取得最佳的长期疗效，但通过分子谱分析识别出PTCL的遗传驱动因素作为治疗靶点已取得了显著进展。针对PTCL的不同亚型量身定制治疗方案可能会带来更个体化的治疗方法，有望改善治疗效果。在本文中，我们综述了目前治疗PTCL的疗法、新发现的分子标志物以及新兴疗法和现有药物新组合的作用。
