Epstein-Barr virus infection with severe consequences. EBV, haemophagocytic lymphohistiocytosis and Hodgkin lymphoma with Down syndrome: EBV, hemofagocytaire lymfohistiocytose en Hodgkin-lymfoom bij Down-syndroom

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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 contributionEpstein-Barr virus infection with severe consequences. EBV, haemophagocytic lymphohistiocytosis and Hodgkin lymphoma with Down syndrome
Original languageDutch
Article numberA7608
JournalNederlands Tijdschrift voor Geneeskunde
Volume158
Publication statusPublished - 2014

Cite this

@article{88b6acff1ab042739ed4034ef95044db,
title = "Een Epstein-Barr-virusinfectie met ernstige gevolgen: EBV, hemofagocytaire lymfohistiocytose en Hodgkin-lymfoom bij Down-syndroom",
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.",
keywords = "Child, Chronic Disease, Down Syndrome/immunology, Epstein-Barr Virus Infections/diagnosis, Hodgkin Disease/complications, Humans, Lymphohistiocytosis, Hemophagocytic/diagnosis, Male, Viral Load",
author = "P.C.J. Kuitert and F.C.H. Abbink and C.J.M. Broers and {van der Valk}, P. and {van Furth}, A.M. and {van der Kuip}, M.",
year = "2014",
language = "Dutch",
volume = "158",
journal = "Nederlands Tijdschrift voor Geneeskunde",
issn = "0028-2162",
publisher = "Bohn Stafleu van Loghum",

}

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 -