BC Cancer Agency, Vancouver, BC.
Hematology Am Soc Hematol Educ Program. 2011;2011:515-24. doi: 10.1182/asheducation-2011.1.515.
Peripheral T-cell lymphomas (PTCLs) are a rare and heterogeneous group of disorders that, for the most part, are associated with a very poor prognosis. The standard therapy for PTCLs is CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) or a comparable CHOP-like regimen that incorporates anthracyclines. With the exception of anaplastic lymphoma kinase-positive anaplastic large cell lymphoma (ALK(+) ALCL), the cure rate for PTCLs with CHOP is low, and limited evidence suggests that anthracyclines do not improve the prognosis. However, there is no compelling evidence that any other regimen or approach is superior. It remains challenging to compare alternative therapies or treatment strategies with CHOP because the majority of data are retrospective and include diverse patient populations. Recently, prospective studies have been initiated exclusively for PTCL, and in some, select histologic subtypes are evaluated in an effort to remove heterogeneity. Encouragingly, there have been several new therapies emerging with activity in PTCLs and exciting novel combinations under consideration that will hopefully move the field forward and improve outcome in this challenging group of diseases.
外周 T 细胞淋巴瘤（PTCLs）是一组罕见且异质性的疾病，它们在很大程度上与非常差的预后相关。PTCLs 的标准治疗是 CHOP（环磷酰胺、多柔比星、长春新碱和泼尼松）或类似的包含蒽环类药物的 CHOP 样方案。除了间变性淋巴瘤激酶阳性间变性大细胞淋巴瘤（ALK(+) ALCL）外，CHOP 治疗 PTCLs 的治愈率较低，并且有限的证据表明蒽环类药物不能改善预后。然而，没有令人信服的证据表明任何其他方案或方法具有优越性。由于大多数数据是回顾性的并且包含不同的患者群体，因此用 CHOP 来比较替代疗法或治疗策略仍然具有挑战性。最近，专门针对 PTCL 启动了前瞻性研究，在某些研究中，选择了特定的组织学亚型进行评估，以消除异质性。令人鼓舞的是，已经出现了几种新的治疗方法，这些方法在外周 T 细胞淋巴瘤中具有活性，并且正在考虑使用一些新的组合，希望这些新方法能够推动该领域的发展并改善这组具有挑战性疾病的预后。
