Severe Epstein-Barr virus-associated hemophagocytic syndrome in six adult patients

J Clin Virol. 2007 Oct;40(2):156-9. doi: 10.1016/j.jcv.2007.06.014. Epub 2007 Aug 20.

Abstract

Background: EBV associated hemophagocytic syndrome (HPS) is an aggressive and potentially life-threatening condition. So far, most EBV associated HPS has been characterized mainly in infants and children in Asian countries.

Results: Here, we report six cases of EBV associated HPS occurring in previously healthy adults in a non-endemic area within a short period of 3 years. All patients presented with fever, hepatosplenomegaly and pancytopenia as well as disturbed liver function tests and coagulopathy. Half were diagnosed as having lymphoma. While EBV-specific serological assays were non-diagnostic in four of the six patients, the presence of EBV DNA in plasma allowed the diagnosis of EBV associated HPS in all patients.

Conclusion: EBV associated HPS may be more prevalent in non-Japanese adults than was previously considered. Screening for hemophagocytic syndrome, in adults as well as in children, should include real-time PCR for EBV.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Antibodies, Viral / blood
  • DNA, Viral / blood
  • Epstein-Barr Virus Infections / complications*
  • Epstein-Barr Virus Infections / diagnosis
  • Female
  • Hepatomegaly / pathology
  • Herpesvirus 4, Human / genetics
  • Herpesvirus 4, Human / immunology
  • Herpesvirus 4, Human / isolation & purification*
  • Humans
  • Liver / physiopathology
  • Lymphohistiocytosis, Hemophagocytic / etiology*
  • Lymphohistiocytosis, Hemophagocytic / pathology
  • Lymphoma / pathology
  • Male
  • Pancytopenia / pathology
  • Splenomegaly / pathology

Substances

  • Antibodies, Viral
  • DNA, Viral