Department of HSCT Data Management and Biostatistics, Graduate School of Medicine, Nagoya University, 1-1-20 Daiko-Minami, Higashi-ku, Nagoya, 461-0047, Japan,
Int J Hematol. 2010 Dec;92(5):697-701. doi: 10.1007/s12185-010-0726-2. Epub 2010 Dec 1.
Extranodal NK/T cell lymphoma, nasal type (ENKL) with advanced stage and aggressive NK-cell leukemia (ANKL) are highly aggressive neoplasms with a dismal clinical outcome. It is well known that P-glycoprotein, which is a product of MDR1 gene and related to multi-drug resistance, is expressed on tumor cells of ENKL or ANKL. This is a major reason for the refractoriness to conventional chemotherapeutic regimens for malignant lymphoma containing anthracycline. However, recent studies have identified that several drugs including L: -asparaginase, methotrexate and alkylators show excellent effect for these tumors. The SMILE (steroid, methotrexate, ifosfamide, L: -asparaginase and etoposide) regimen is one of the promising regimens for advanced or relapsed/refractory ENKL, but its myelotoxicity is strong. ANKL needs another treatment strategy because of a systemic disease progression and extensive organ insufficiency. Optimal treatment scheme using such effective agents for these unfavorable NK-cell tumors should further be explored.
结外 NK/T 细胞淋巴瘤，鼻型（ENKL）伴晚期侵袭性 NK 细胞白血病（ANKL）是具有极差临床结局的高度侵袭性肿瘤。众所周知，多药耐药基因（MDR1）产物 P-糖蛋白表达于 ENKL 或 ANKL 肿瘤细胞，与多药耐药相关，这是导致含蒽环类药物的恶性淋巴瘤常规化疗方案耐药的主要原因。然而，最近的研究表明，包括 L: -天冬酰胺酶、甲氨蝶呤和烷化剂在内的几种药物对这些肿瘤显示出良好的效果。SMILE（类固醇、甲氨蝶呤、异环磷酰胺、L: -天冬酰胺酶和依托泊苷）方案是晚期或复发/难治性 ENKL 的一种有前途的方案，但骨髓抑制作用较强。由于全身性疾病进展和广泛的器官功能不全，ANKL 需要另一种治疗策略。对于这些不良 NK 细胞肿瘤，应该进一步探索使用这些有效药物的最佳治疗方案。
