Hôpital Saint Louis, Institut d'hématologie Paris Diderot University.
Hematology Department, Necker University Hospital, AP-HP, Paris Descartes University - Sorbonne Paris Cité, Paris, France.
Curr Opin Oncol. 2018 Sep;30(5):285-291. doi: 10.1097/CCO.0000000000000469.
Peripheral T-cell lymphoma (PTCL) is a heterogeneous group of mature T-cell and natural killer (NK)-cell neoplasms in the WHO 2016 classification. Patient prognosis is poor when treated with CHOP, and there is an unmet need for new drugs. Several agents have been developed for PTCL, and their use is the subject of this review.
Phase 2 studies demonstrated the activity of new drugs in Relapsed/refractory PTCL. Only four compounds were approved by the food and drug administration: romidepsin and belinostat, which are epigenetic modifiers, the antifolate agent pralatrexate, the immuno-conjugate brentuximab vedotin. New combinations have been tested, but the results were disappointing. Given the latest progress in biology, targeted agents are evaluated in different subtypes of PTCL. Relapsed anaplastic large-cell lymphoma exhibits improved prognosis with the approved anti-CD30 drug conjugate brentuximab vedotin. Localized nasal NK/T is treated with radiotherapy and nonanthracycline chemotherapy with L-asparaginase. Recently, immune checkpoint inhibitors demonstrated activity in NK/T lymphoma and can be used in elderly patients.
Treatment remains a challenge for PTCL, and several targeted drugs provide new approaches. Progress will be made incrementally in the different subtypes. One of the critical situations facing new drugs is the ability to run robust clinical trials in rare diseases.
外周 T 细胞淋巴瘤（PTCL）是世界卫生组织 2016 年分类中的一组成熟 T 细胞和自然杀伤（NK）细胞肿瘤的异质性群体。当用 CHOP 治疗时，患者预后较差，因此需要新的药物。已经开发了几种用于 PTCL 的药物，本文将对此进行综述。
在复发/难治性 PTCL 的 2 期研究中，新型药物显示出活性。仅有四种化合物被食品和药物管理局批准：组蛋白去乙酰化酶抑制剂罗米地辛和贝利司他、叶酸类似物普拉曲沙、免疫偶联药物 Brentuximab vedotin。已测试了新的组合，但结果令人失望。鉴于生物学的最新进展，靶向药物在不同类型的 PTCL 中进行了评估。已批准的抗 CD30 药物偶联物 Brentuximab vedotin 可改善复发间变大细胞淋巴瘤的预后。局部鼻 NK/T 细胞淋巴瘤采用放射治疗和不含蒽环类药物的化疗联合 L-天冬酰胺酶治疗。最近，免疫检查点抑制剂在 NK/T 淋巴瘤中显示出活性，可用于老年患者。
PTCL 的治疗仍然是一个挑战，几种靶向药物提供了新的方法。在不同的亚型中，将逐步取得进展。新型药物面临的一个关键情况是能否在罕见疾病中进行稳健的临床试验。
