Natural killer-cell neoplasms.
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 NK-cell neoplasms include immature acute leukemias; a blastic NK-cell lymphoma, which is obsolete because of its plasmacytoid dendritic-cell origin; and mature NK neoplasms, comprising extranodal NK/T-cell lymphoma (ENKL), nasal-type; aggressive NK-cell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.
Greer
John P
JP
Hematology/Stem Cell Transplantation, 1301 Medical Center Drive, 2665 TVC, Nashville, TN 37232, USA. john.greer@Vanderbilt.Edu
Mosse
Claudio A
CA
D000970:Antineoplastic Agents / Q000627:therapeutic use; D019070:Cell Lineage; D002471:Cell Transformation, Neoplastic; D006801:Humans; D007694:Killer Cells, Natural / Q000473:pathology*; D007938:Leukemia / Q000473:pathology* / Q000628:therapy; D008223:Lymphoma / Q000473:pathology* / Q000628:therapy; D008232:Lymphoproliferative Disorders / Q000473:pathology* / Q000628:therapy; D033581:Stem Cell Transplantation
D000970:Antineoplastic Agents