Een Epstein-Barr-virusinfectie met ernstige gevolgen

EBV, hemofagocytaire lymfohistiocytose en Hodgkin-lymfoom bij Down-syndroom
Casuïstiek
20-06-2014
Pieter C.J. Kuitert, Floor C.H. Abbink, Chantal J.M. Broers, Paul van der Valk, A. Marceline van Furth en Martijn van der Kuip

Epstein-Barr virus infection with severe consequences. EBV, haemophagocytic lymphohistiocytosis and Hodgkin lymphoma with Down syndrome

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).

Case description

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.

Conclusion

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.

Conflict of interest: none declared. Financial support: none declared.