title: Nasal angiocentric lymphoma with hemophagocytic syndrome.
abstract: OBJECTIVE
  Hemophagocytic syndrome (HS) is a fatal complication of nasal angiocentric lymphoma (AL) and difficult to distinguish from malignant histiocyosis. Epstein-Barr virus (EBV)-associated HS is frequently observed in lymphoma of T-cell lineage and EBV is highly associated with nasal AL. Clinicopathologic features of 10 nasal ALs with HS were reviewed to determine the clinical significance and the pathogenetic association with EBV.
  
  
  METHODS
  Ten patients of HS were identified from a retrospective analysis of 42 nasal ALs diagnosed from 1987 to 1996. Immunohistochemical study and in situ hybridization were performed on the paraffin-embedded tumor specimens obtained from 10 patients. Serologic study of EBV-Ab was performed in 3 available patients.
  
  
  RESULTS
  Five patients had HS as initial manifestation, 3 at the time of relapse and 2 during the clinical remission of AL. Four patients were treated by combination chemotherapy (CHOP) and others had only supportive care. The median survival of all patients with HS was 4.1 months (range 2 days-36.5 months) and all had fatal outcome regardless of the treatment-modality. All cases were positive for UCHL1 (CD45RO) and EBV by EBER in situ hybridization. The data of serologic tests indicated the active EBV infection.
  
  
  CONCLUSIONS
  HS is a fatal complication of nasal AL and has a high association with EBV. Reactivation of EBV may contribute to HS and further investigation of predictive factors and effective treatment of HS should be pursued in the future.
authors.affiliation: Department of Internal Medicine, Catholic University of Korea College of Medicine, Seoul, Korea.
authors.forename: J Y
authors.initials: JY
authors.lastname: Han
authors.forename: E J
authors.initials: EJ
authors.lastname: Seo
authors.forename: H J
authors.initials: HJ
authors.lastname: Kwon
authors.forename: K O
authors.initials: KO
authors.lastname: Min
authors.forename: J S
authors.initials: JS
authors.lastname: Kim
authors.forename: J H
authors.initials: JH
authors.lastname: Kang
authors.forename: Y S
authors.initials: YS
authors.lastname: Hong
authors.forename: H K
authors.initials: HK
authors.lastname: Kim
authors.forename: K S
authors.initials: KS
authors.lastname: Lee
mesh_terms: D000328:Adult; D020031:Epstein-Barr Virus Infections / Q000150:complications; D005260:Female; D015616:Histiocytosis, Non-Langerhans-Cell / Q000150:complications* / Q000473:pathology; D006801:Humans; D008223:Lymphoma / Q000150:complications* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009669:Nose Neoplasms / Q000150:complications* / Q000473:pathology; D013577:Syndrome