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).
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.
| Translated title of the contribution | Epstein-Barr virus infection with severe consequences. EBV, haemophagocytic lymphohistiocytosis and Hodgkin lymphoma with Down syndrome |
|---|---|
| Original language | Dutch |
| Article number | A7608 |
| Journal | Nederlands Tijdschrift voor Geneeskunde |
| Volume | 158 |
| Publication status | Published - 2014 |
Cite this
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Een Epstein-Barr-virusinfectie met ernstige gevolgen : EBV, hemofagocytaire lymfohistiocytose en Hodgkin-lymfoom bij Down-syndroom. / Kuitert, P.C.J.; Abbink, F.C.H.; Broers, C.J.M.; van der Valk, P.; van Furth, A.M.; van der Kuip, M.
In: Nederlands Tijdschrift voor Geneeskunde, Vol. 158, A7608, 2014.Research output: Contribution to journal › Article › Professional
TY - JOUR
T1 - Een Epstein-Barr-virusinfectie met ernstige gevolgen
T2 - EBV, hemofagocytaire lymfohistiocytose en Hodgkin-lymfoom bij Down-syndroom
AU - Kuitert, P.C.J.
AU - Abbink, F.C.H.
AU - Broers, C.J.M.
AU - van der Valk, P.
AU - van Furth, A.M.
AU - van der Kuip, M.
PY - 2014
Y1 - 2014
N2 - 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.
AB - 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.
KW - Child
KW - Chronic Disease
KW - Down Syndrome/immunology
KW - Epstein-Barr Virus Infections/diagnosis
KW - Hodgkin Disease/complications
KW - Humans
KW - Lymphohistiocytosis, Hemophagocytic/diagnosis
KW - Male
KW - Viral Load
M3 - Article
VL - 158
JO - Nederlands Tijdschrift voor Geneeskunde
JF - Nederlands Tijdschrift voor Geneeskunde
SN - 0028-2162
M1 - A7608
ER -