title: Diagnosis and management of extranodal NK/T cell lymphoma nasal type.
abstract: BACKGROUND
  Extranodal NK/T-cell lymphoma nasal type is a distinct clinicopathologic entity. The most common initial site of presentation is the nasopharyngeal area, but non-nasals sites including the skin and the gastrointestinal tract may be affected.
  
  
  METHODS
  The diagnosis and management of NK/T-cell lymphoma is discussed, based on a literature search on PubMed. NK/T-cell lymphoma are typically positive for CD3 (cytoplasmic), CD56, cytotoxic markers (granzyme B, TIA1) and Epstein Barr virus (EBV). Plasma EBV DNA is an accurate surrogate biomarker for lymphoma load. For stage I/II nasal lymphoma, a combination of chemotherapy and radiotherapy yields the best results. Concomitant chemoradiotherapy and sequential chemotherapy and radiotherapy give similar response rates and survivals. For stage III/IV nasal lymphoma and non-nasal lymphomas, chemotherapy is the mainstay of treatment. Conventional anthracycline-based regimens are ineffective. Recommended chemotherapy protocols are based on the use of L-asparaginase combined with other effective drugs. Durable remission can be expected in at least 60% of patients irrespective of stage. Prognostically models based on clinicopathologic parameters and EBV DNA load are useful in stratification of patients for therapy. Expert commentary: Current treatment leads to long-term survival in a significant proportion of patients. For relapsed patients, novel strategies are needed.
authors.affiliation: a Department of Medicine , Queen Mary Hospital , Hong Kong , China.
authors.forename: Eric
authors.initials: E
authors.lastname: Tse
authors.affiliation: a Department of Medicine , Queen Mary Hospital , Hong Kong , China.
authors.forename: Yok-Lam
authors.initials: YL
authors.lastname: Kwong
mesh_terms: D059248:Chemoradiotherapy; D003131:Combined Modality Therapy; D019468:Disease Management; D019008:Drug Resistance, Neoplasm; D020031:Epstein-Barr Virus Infections / Q000150:complications / Q000821:virology; D020022:Genetic Predisposition to Disease; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D016130:Immunophenotyping; D007694:Killer Cells, Natural / Q000378:metabolism / Q000473:pathology; D054391:Lymphoma, Extranodal NK-T-Cell / Q000175:diagnosis* / Q000209:etiology / Q000401:mortality / Q000628:therapy*; D064847:Multimodal Imaging; D009154:Mutation; D009367:Neoplasm Staging; D010641:Phenotype; D011379:Prognosis; D012008:Recurrence; D016896:Treatment Outcome
keywords: Epstein Barr virus; Extranodal NK/T-cell lymphoma; L-asparaginase; hematopoietic stem cell transplantation; natural killer cell