Chaudhary Ranjit Kumar, Bhatt Vijaya Raj, Vose Julie M
Department of Medicine, Institute of Medicine, Tribhuvan University, Kathmandu, Nepal.
Division of Hematology and Oncology, Department of Internal Medicine, University of Nebraska Medical Center, Omaha, NE.
Clin Lymphoma Myeloma Leuk. 2015 May;15(5):245-52. doi: 10.1016/j.clml.2014.12.014. Epub 2015 Jan 3.
Extranodal natural killer/T-cell lymphoma, nasal type (NKTL) is an uncommon aggressive subtype of non-Hodgkin lymphoma, which is significantly more common in East Asia and Latin America. Three-quarters of patients present with stage I/II disease, and half of patients have a low-risk International Prognostic Index score. Although additional factors including natural killer/T-cell lymphoma prognostic index and peripheral blood Epstein-Barr virus load influence the outcomes, the modality of treatment largely differs according to stage (based on nonrandomized studies). In early-stage disease, a combination of chemotherapy and involved-field radiotherapy (IFRT) appears to improve outcome compared with chemotherapy alone. Radiotherapy dose > 50 Gy, concurrent or sequential chemoradiation, and early use of IFRT results in better outcomes than doses < 50 Gy and delay in initiation of IFRT. In late-stage NKTL, chemotherapy alone is the mainstay of treatment. Compared with the CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) regimen, newer regimens containing L-asparaginase and gemcitabine result in better outcomes; however, toxicity might be an issue. Stem cell transplant has been used as salvage and consolidation therapy with some benefit, particularly in patients with advanced-stage disease in remission at the time of transplant; however, further confirmatory studies are needed. In the past decade, we have seen a significant growth in our understanding of the disease process and an increase in our armamentarium of effective therapeutics; however, further development of novel therapies and optimal selection of available therapy options via randomized trials are necessary to improve the outcomes of this aggressive lymphoma.
结外自然杀伤细胞/T细胞淋巴瘤，鼻型（NKTL）是一种罕见的侵袭性非霍奇金淋巴瘤亚型，在东亚和拉丁美洲更为常见。四分之三的患者表现为Ⅰ/Ⅱ期疾病，半数患者的国际预后指数评分低。尽管包括自然杀伤细胞/T细胞淋巴瘤预后指数和外周血EB病毒载量等其他因素会影响预后，但治疗方式在很大程度上因分期而异（基于非随机研究）。在早期疾病中，与单纯化疗相比，化疗联合受累野放疗（IFRT）似乎能改善预后。放疗剂量>50 Gy、同步或序贯放化疗以及早期使用IFRT比剂量<50 Gy和延迟开始IFRT能带来更好的预后。在晚期NKTL中，单纯化疗是主要的治疗方法。与CHOP（环磷酰胺、阿霉素、长春新碱和泼尼松）方案相比，含L-天冬酰胺酶和吉西他滨的新方案能带来更好的预后；然而，毒性可能是个问题。干细胞移植已被用作挽救和巩固治疗，有一定益处，特别是对于移植时处于缓解期的晚期疾病患者；然而，还需要进一步的验证性研究。在过去十年中，我们对疾病过程的理解有了显著增长，有效治疗手段也有所增加；然而，通过随机试验进一步开发新疗法并优化现有治疗方案的选择对于改善这种侵袭性淋巴瘤的预后是必要的。
