Ma Helen, Marchi Enrica, O'Connor Owen A
Section of Hematology and Oncology, Long Beach Veterans Administration Healthcare System, Long Beach, CA, USA.
Division of Hematology and Oncology, Program for T-Cell Lymphoma Research, University of Virginia Cancer Center, Charlottesville, VA, USA.
Lancet Haematol. 2020 Oct;7(10):e765-e771. doi: 10.1016/S2352-3026(20)30207-6.
Over the past 30 years, the scientific community has made little progress in changing the natural history of peripheral T-cell lymphomas. Of the haematological malignancies, T-cell lymphomas have an extremely poor prognosis. One reason for this poor outcome has been that no treatment programme has ever been developed specifically for the broader category of the disease-peripheral T-cell lymphoma-let alone any of the specific subtypes, except advances made for patients with CD30-positive anaplastic large cell lymphoma. Decades of effort have focused on retrofitting chemotherapy programmes used for other diseases, such as CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) for diffuse large B-cell lymphoma, which have not been associated with much progress, and have universally produced far more toxicity than benefit. A remarkable heterogeneity, a paucity of cases, and the absence of peripheral T-cell lymphoma-specific drugs, until recently at least, have limited the field's ability to make substantive and innovative advances. Over the past few years, however, it appears the field is beginning to make progress. Lineage and disease-specific novel-to-novel platforms are producing, although perhaps not unsurprisingly, compelling results suggesting that the path to a cure for this rare orphan disease might be heading in a different direction.
在过去30年里,科学界在改变外周T细胞淋巴瘤的自然病程方面进展甚微。在血液系统恶性肿瘤中,T细胞淋巴瘤的预后极差。预后不佳的一个原因是,从未专门针对外周T细胞淋巴瘤这一宽泛的疾病类别制定过治疗方案,更不用说任何特定亚型了,除了针对CD30阳性间变性大细胞淋巴瘤患者取得的进展。数十年来的努力都集中在对用于其他疾病的化疗方案进行改造,比如用于弥漫性大B细胞淋巴瘤的CHOP方案(环磷酰胺、阿霉素、长春新碱和泼尼松),但这些方案并未带来太大进展,而且普遍产生的毒性远远超过益处。显著的异质性、病例数量稀少以及缺乏外周T细胞淋巴瘤特异性药物,至少直到最近,都限制了该领域取得实质性和创新性进展的能力。然而,在过去几年里,该领域似乎开始取得进展。谱系和疾病特异性的新型到新型平台正在产生,尽管也许并不令人意外,令人信服的结果表明,治愈这种罕见的孤儿疾病的道路可能正朝着不同的方向发展。