title: Cutaneous intravascular natural killer-cell lymphoma: a case report and review of the literature.
abstract: OBJECTIVE
  To our knowledge, since 2003, there have been 11 reported cases of intravascular natural killer (NK)-cell lymphoma (IVNKL). Herein we describe the 12th case.
  
  
  METHODS
  H&E and Envision immunohistochemical stains as well as in situ hybridization were used to study this disease in combination with review of the literature.
  
  
  RESULTS
  Half of the cases reported to date are from China and Taipei. The clinical manifestation of IVNKL is erythema in the limbs and trunk, although patients' conditions have varied notably from each other. One-year survival rate is about 40%.
  
  
  CONCLUSIONS
  IVNKL should be distinguished from extranodal NK/T-cell lymphoma (nasal type) and aggressive NK-cell leukemia. These three diseases have a similar phenotype and are all related to Epstein-Barr virus infection. However, the pathogenesis of similarities and differences needs further study. In particular, IVNKL is quite unusual. The treatment of IVNKL is difficult, and the prognosis is poor. Currently, IVNKL is not included in the World Health Organization classification subtypes and has been classified into NK/T-cell lymphoma (nasal type). However, in view of the unique characteristics of this disease, we propose that the diagnosis be independent, since this will facilitate further study of this disease.
authors.affiliation: From the Department of Pathology, Bethune International Peace Hospital, Shijiazhuang, Hebei Province, China.
authors.forename: Yanli
authors.initials: Y
authors.lastname: Liu
authors.affiliation: From the Department of Pathology, Bethune International Peace Hospital, Shijiazhuang, Hebei Province, China.
authors.forename: Wen
authors.initials: W
authors.lastname: Zhang
authors.affiliation: From the Department of Pathology, Bethune International Peace Hospital, Shijiazhuang, Hebei Province, China.
authors.forename: Jie
authors.initials: J
authors.lastname: An
authors.affiliation: From the Department of Pathology, Bethune International Peace Hospital, Shijiazhuang, Hebei Province, China.
authors.forename: Hui
authors.initials: H
authors.lastname: Li
authors.affiliation: From the Department of Pathology, Bethune International Peace Hospital, Shijiazhuang, Hebei Province, China. liu_shuang@hotmail.com.
authors.forename: Shuang
authors.initials: S
authors.lastname: Liu
mesh_terms: D000328:Adult; D001706:Biopsy / Q000379:methods; D020031:Epstein-Barr Virus Infections / Q000150:complications / Q000473:pathology*; D005260:Female; D006801:Humans; D007694:Killer Cells, Natural / Q000378:metabolism / Q000473:pathology*; D016410:Lymphoma, T-Cell, Cutaneous / Q000378:metabolism / Q000401:mortality* / Q000473:pathology* / Q000821:virology; D012878:Skin Neoplasms / Q000175:diagnosis / Q000401:mortality* / Q000473:pathology* / Q000821:virology; D015996:Survival Rate
keywords: Intravascular lymphoma; Natural killer (NK)–cell lymphoma; Poor prognosis; Subcutaneous lesions