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

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.

Publication types

  • Review

MeSH terms

  • Antineoplastic Agents / administration & dosage
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use*
  • Asparaginase / administration & dosage
  • Asparaginase / therapeutic use
  • Dexamethasone / administration & dosage
  • Drug Therapy, Combination
  • Etoposide / administration & dosage
  • Humans
  • Ifosfamide / administration & dosage
  • Killer Cells, Natural / pathology*
  • Leukemia, T-Cell / therapy*
  • Lymphoma, Extranodal NK-T-Cell / drug therapy*
  • Lymphoma, Extranodal NK-T-Cell / pathology
  • Methotrexate / administration & dosage
  • Treatment Outcome


  • Antineoplastic Agents
  • Etoposide
  • Dexamethasone
  • Asparaginase
  • Ifosfamide
  • Methotrexate