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Natural killer-cell neoplasms

Abstract

The natural killer (NK)-cell neoplasms are rare, representing less than 1% of non-Hodgkin lymphoma, except in Asia and Latin America, where they represent 3% to 6%. NK-cell neoplasms include immature acute leukemias; a blastic NK-cell lymphoma, which is obsolete because of its plasmacytoid dendritic-cell origin; and mature NK neoplasms, comprising extranodal NK/T-cell lymphoma (ENKL), nasal-type; aggressive NK-cell leukemia; and chronic NK-cell lymphoproliferative disorders, which are often reactive. Epstein-Barr virus is usually detected in tumor cells of ENKL and aggressive NK-cell leukemia. The latter two mature NK neoplasms are relatively chemoresistant because of the frequent expression of P-glycoprotein. Early radiation is advocated for localized nasal ENKL. Stem cell transplantation is recommended for advanced disease, owing to a poor prognosis. Novel agents, including chemotherapy, inhibitors of molecular pathways, and monoclonal antibodies, are under investigation.

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Correspondence to John P. Greer.

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Greer, J.P., Mosse, C.A. Natural killer-cell neoplasms. Curr Hematol Malig Rep 4, 245–252 (2009). https://doi.org/10.1007/s11899-009-0032-3

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Keywords

  • Natural Killer
  • Natural Killer Cell
  • Alemtuzumab
  • Large Granular Lymphocyte
  • Pralatrexate