International Journal of Hematology

, Volume 92, Issue 5, pp 697–701 | Cite as

Treatment of advanced extranodal NK/T cell lymphoma, nasal-type and aggressive NK-cell leukemia

  • Ritsuro SuzukiEmail author
Progress in Hematology New insights in NK-cell malignancies


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.


Natural killer cell lymphoma Treatment l-asparaginase 


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Copyright information

© The Japanese Society of Hematology 2010

Authors and Affiliations

  1. 1.Department of HSCT Data Management and Biostatistics, Graduate School of MedicineNagoya UniversityNagoyaJapan

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