Een Epstein-Barr-virusinfectie met ernstige gevolgen: EBV, hemofagocytaire lymfohistiocytose en Hodgkin-lymfoom bij Down-syndroom

Autor: Kuitert, P.C.J., Abbink, F.C.H., Broers, C.J.M., van der Valk, P., van Furth, A.M., van der Kuip, M.
Přispěvatelé: Pediatric surgery, Pathology, CCA - Oncogenesis
Jazyk: Dutch; Flemish
Rok vydání: 2014
Předmět:
Zdroj: Nederlands Tijdschrift voor Geneeskunde, 158:A7608. Bohn Stafleu van Loghum
ISSN: 0028-2162
Popis: 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.
Databáze: OpenAIRE