title: [Epstein-Barr virus infection with severe consequences. EBV, haemophagocytic lymphohistiocytosis and Hodgkin lymphoma with Down syndrome].
abstract: BACKGROUND
  Over 90% of the population is infected with the Epstein-Barr virus (EBV). Following primary infection, the virus remains latent in B-lymphocytes. In isolated cases, especially in immunocompromised patients, the Epstein-Barr virus can result in a chronic active infection (CAEBV).
  
  
  METHODS
  We describe an 11-year-old boy with Down syndrome who was admitted because of fever of unknown origin during several periods. Serological findings (high VCA-IgG and absent EBNA-IgG) were suggestive of CAEBV, which was confirmed by the high circulating EBV viral load. During admission the clinical picture worsened and our patient developed pancytopenia, which led us to diagnose concurrent haemophagocytic lymphohistiocytosis and Hodgkin lymphoma.
  
  
  CONCLUSIONS
  CAEBV is the result of deficiency in cellular immunity, which in this patient could possibly be attributed to deficiencies in the immune system associated with Down syndrome. CAEBV is difficult to treat, and it can come with life-threatening complications such as haemophagocytic lymphohistiocytosis.
authors.affiliation: VU medisch centrum, afd. Kindergeneeskunde, Amsterdam.
authors.forename: Pieter C J
authors.initials: PC
authors.lastname: Kuitert
authors.forename: Floor C H
authors.initials: FC
authors.lastname: Abbink
authors.forename: Chantal J M
authors.initials: CJ
authors.lastname: Broers
authors.forename: Paul
authors.initials: P
authors.lastname: van der Valk
authors.forename: A Marceline
authors.initials: AM
authors.lastname: van Furth
authors.forename: Martijn
authors.initials: M
authors.lastname: van der Kuip
mesh_terms: D002648:Child; D002908:Chronic Disease; D004314:Down Syndrome / Q000276:immunology*; D020031:Epstein-Barr Virus Infections / Q000175:diagnosis*; D006689:Hodgkin Disease / Q000150:complications / Q000175:diagnosis*; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000175:diagnosis*; D008297:Male; D019562:Viral Load