Department of Oncology and Hematology, Shimane University Hospital Cancer Center, 89-1 En-ya cho, Izumo, 693-8501, Japan.
Curr Hematol Malig Rep. 2018 Feb;13(1):7-12. doi: 10.1007/s11899-018-0430-5.
Extranodal NK/T cell lymphoma (ENKL), nasal type, is a highly aggressive lymphoma which used to show a poor clinical outcome. Expression of P-glycoprotein on lymphoma cells of ENKL is a major reason for the refractoriness to conventional chemotherapy containing anthracycline. However, recent innovative approaches have improved the outcome and prognosis of ENKL. The purpose of this review is to summarize the proceedings of treatment.
Concurrent chemoradiotherapy containing platinum and several drugs including L-asparaginase, methotrexate, and alkylators shows excellent outcomes for the limited-stage ENKL. SMILE (steroid, methotrexate, ifosfamide, L-asparaginase, and etoposide) or other L-asparaginase-containing therapy is promising for advanced-stage ENKL, followed by either autologous or allogeneic hematopoietic stem cell transplantation. Anti-PD-1 or other immunological checkpoint inhibitors are recently reported to be effective for relapsed/refractory ENKL thought to be due to EBV-driven upregulation of PD-L1 expression. The prognosis of ENKL is therefore improving by the introduction of these strategies. The 5-year overall survival (OS) rate of limited stage was 63.2% [95% confidence interval (CI), 55.3 to 70.0%] before 2010, but was 79.4% (95% CI, 66.9 to 87.6%) in 2010 or after. However, there still exists a room for improvement, particularly for advanced-stage patients. The 2-year OS of advanced ENKL was 30.3% (95% CI, 19.5 to 41.7%) before 2010, but was 40.5% (95% CI, 24.8 to 55.8%) in 2010 or after. Optimal treatment scheme should further be explored.
结外 NK/T 细胞淋巴瘤（ENKL），鼻型，是一种侵袭性很强的淋巴瘤，对包含蒽环类药物的常规化疗具有耐药性，导致其临床预后较差。ENKL 淋巴瘤细胞表达 P-糖蛋白是其对常规包含蒽环类化疗耐药的主要原因。然而，最近的创新方法改善了 ENKL 的疗效和预后。本文旨在总结其治疗进展。
含铂类药物和几种药物（包括 L-天冬酰胺酶、甲氨蝶呤和烷化剂）的同期放化疗对局限性 ENKL 疗效显著。SMILE（类固醇、甲氨蝶呤、异环磷酰胺、L-天冬酰胺酶和依托泊苷）或其他含 L-天冬酰胺酶的治疗方案对晚期 ENKL 有较好的疗效，随后进行自体或异基因造血干细胞移植。抗 PD-1 或其他免疫检查点抑制剂最近被报道对复发/难治性 ENKL 有效，这可能是由于 EBV 驱动 PD-L1 表达上调所致。由于这些策略的引入，ENKL 的预后得到了改善。2010 年之前局限性 ENKL 的 5 年总生存率（OS）为 63.2%（95%CI，55.3%至 70.0%），2010 年或之后则为 79.4%（95%CI，66.9%至 87.6%）。然而，仍有改善的空间，特别是对晚期患者。2010 年之前，晚期 ENKL 的 2 年 OS 为 30.3%（95%CI，19.5%至 41.7%），2010 年或之后则为 40.5%（95%CI，24.8%至 55.8%）。应进一步探索最佳治疗方案。
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