Institute of Hematology "L. e A. Seràgnoli," University of Bologna, Bologna, Italy.
Blood. 2017 Mar 2;129(9):1103-1112. doi: 10.1182/blood-2016-08-692566. Epub 2017 Jan 23.
Peripheral T-cell lymphoma, not otherwise specified, is a broad category of biologically and clinically heterogeneous diseases that cannot be further classified into any other of the existing entities defined by the World Health Organization classification. Anthracycline-containing regimens, namely cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP), nowadays represent the standard first-line treatment; for patients who achieve a satisfactory response, a consolidation by means of autologous stem cell transplantation may offer a greater chance of long-term survival. Several patients, however, display treatment refractoriness or relapse soon after obtaining a response, and just a few of them are suitable transplant candidates. This is why several new agents, with innovative mechanisms of action, have been investigated in this context: pralatrexate, romidepsin, belinostat, and brentuximab vedotin have been approved for relapsed and refractory peripheral T-cell lymphomas based on their activity, although they do not significantly affect survival rates. The incorporation of such new drugs within a CHOP backbone is under investigation to enhance response rates, allow a higher proportion of patients to be transplanted in remission, and prolong survival.
未特指的外周 T 细胞淋巴瘤是一类生物学和临床异质性很强的疾病，无法进一步归入世界卫生组织分类定义的任何其他现有实体。含蒽环类药物的方案，即环磷酰胺、多柔比星、长春新碱和泼尼松（CHOP），如今代表了标准的一线治疗；对于获得满意缓解的患者，自体干细胞移植巩固治疗可能会提供更高的长期生存机会。然而，许多患者在获得缓解后表现出治疗耐药或复发，而且只有少数患者适合移植。这就是为什么在这种情况下研究了几种具有创新作用机制的新型药物：普拉曲沙、罗米地辛、贝林司他和本妥昔单抗维达妥因已基于其活性被批准用于复发和难治性外周 T 细胞淋巴瘤，尽管它们并未显著影响生存率。正在研究在 CHOP 基础上加入这些新型药物以提高缓解率、使更多的患者在缓解期接受移植并延长生存期。
