Stanford Cancer Center, 875 Blake Wilbur Drive, MC5151, Stanford, CA 94305, USA.
Expert Rev Anticancer Ther. 2010 Sep;10(9):1395-405. doi: 10.1586/era.10.130.
Extranodal natural killer/T-cell lymphoma (ENKL), a subtype of natural killer/T-cell malignancies, is a rare subset of lymphomas with significant biological and clinical heterogeneity. The prognosis of ENKL is variable and therapeutic approaches are not well established. The optimal dose, combination, and sequence of radiotherapy and chemotherapy are evolving, as is the role of stem cell transplantation. Radiotherapy is an essential component of therapy for early-stage disease. The clinical course of advanced disease is highly aggressive, with frequent chemotherapy resistance and a poor prognosis. For relapsed disease, asparaginase-based regimens have provided encouraging results and are currently under investigation in the frontline setting. Our article discusses the key aspects of biology, pathogenesis and clinical presentation that contribute to the heterogeneity, and proposes a stratified approach to the treatment of ENKL based on clinical, pathologic and biologic risk factors. Although considerable advances have been made in our understanding of the biology and prognosis of this lymphoma, it remains critical that all patients with a diagnosis of ENKL are enrolled and treated in clinical trials so that optimal therapies can be identified.
结外自然杀伤/T 细胞淋巴瘤（ENKL）是自然杀伤/T 细胞恶性肿瘤的一个亚型，是具有显著生物学和临床异质性的淋巴瘤的一个罕见亚群。ENKL 的预后各不相同，治疗方法尚未确定。放疗和化疗的最佳剂量、组合和顺序正在不断发展，造血干细胞移植的作用也是如此。放疗是早期疾病治疗的重要组成部分。晚期疾病的临床病程具有侵袭性，经常发生化疗耐药和预后不良。对于复发疾病，基于天冬酰胺酶的方案提供了令人鼓舞的结果，目前正在一线治疗中进行研究。我们的文章讨论了导致异质性的生物学、发病机制和临床表现的关键方面，并根据临床、病理和生物学危险因素提出了针对 ENKL 的分层治疗方法。尽管我们对这种淋巴瘤的生物学和预后有了相当大的了解，但关键是所有诊断为 ENKL 的患者都要在临床试验中登记和治疗，以便确定最佳治疗方法。
