Department of Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL.
Division of Hematology and Oncology, Northwestern University Feinberg School of Medicine and Robert H Lurie Comprehensive Cancer Center, Chicago, IL.
Semin Hematol. 2021 Apr;58(2):85-94. doi: 10.1053/j.seminhematol.2021.02.006. Epub 2021 Mar 6.
Peripheral T-cell lymphomas (PTCL) are rare lymphoproliferative disorders with poor outcomes and high rates of relapse. Incidence varies although the most common subtypes include PTCL-not-otherwise specified, anaplastic large cell lymphoma, and angioimmunoblastic T-cell lymphoma. Anaplastic large cell lymphoma is characterized by near-universal CD30 expression and serves as a prototypic model for other CD30-expressing lymphomas. Historically, these neoplasms have been treated with regimens used in the treatment of aggressive B-cell lymphomas. Over the last decade, brentuximab vedotin, an antibody-drug conjugate, has been investigated to treat peripheral T-cell lymphomas expressing CD30. While first studied in the relapsed and refractory setting, it was later studied in the frontline setting in the ECHELON-2 trial with positive results and is now an approved treatment for CD30-expressing peripheral T-cell lymphomas. Other treatment options in the relapsed and refractory setting include histone deacetylase inhibitors, pralatrexate, and salvage multiagent chemotherapy regimens. Current research is underway regarding combination therapies and the use of other novel agents.
外周 T 细胞淋巴瘤（PTCL）是一种罕见的淋巴增生性疾病，预后不良，复发率高。发病率有所不同，尽管最常见的亚型包括未特指的外周 T 细胞淋巴瘤、间变大细胞淋巴瘤和血管免疫母细胞性 T 细胞淋巴瘤。间变大细胞淋巴瘤的特征是几乎普遍表达 CD30，是其他表达 CD30 的淋巴瘤的典型模型。从历史上看，这些肿瘤采用治疗侵袭性 B 细胞淋巴瘤的方案进行治疗。在过去的十年中，抗体药物偶联物 Brentuximab vedotin 已被用于治疗表达 CD30 的外周 T 细胞淋巴瘤。虽然最初在复发和难治性环境中进行了研究，但后来在 ECHELON-2 试验的一线环境中进行了研究，结果为阳性，现在已被批准用于治疗表达 CD30 的外周 T 细胞淋巴瘤。在复发和难治性环境中的其他治疗选择包括组蛋白去乙酰化酶抑制剂、普拉曲沙和挽救性多药化疗方案。目前正在进行关于联合治疗和使用其他新型药物的研究。
