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Acta Paediatr. 2003 Jul;92(7):859-61.

Epstein-Barr virus-associated haemophagocytic lymphohistiocytosis in Wiskott-Aldrich syndrome.

Author information

1
Department of Paediatric Immunology, Mother and Child Health Institute, Belgrade, Yugoslavia. bg.pasic@eunet.yu

Abstract

A patient with Wiskott-Aldrich syndrome who developed Epstein-Barr virus-associated haemophagocytic lymphohistiocytosis (EBV-HLH) is described in this study. At 4 mo of age the patient developed fever associated with bicytopenia and splenomegaly. Analysis of a bone marrow specimen revealed extensive haemophagocytosis, and in situ hybridization for EBV of the bone marrow specimen using an EBV-encoded RNA probe was positive. Diagnosis of EBV-HLH was established and immunotherapy with HLH-94 protocol was started. HLH has been described in patients with other well-defined primary immunodeficiencies such as X-linked lymphoproliferative syndrome, Chediak-Higashi syndrome and Griscelli disease. Also, HLH was reported recently in severe combined immunodeficiency and DiGeorge syndrome.

CONCLUSION:

The possibility of an underlying primary immunodeficiency should be considered in paediatric patients who present with HLH during infancy.

PMID:
12892170
DOI:
10.1080/08035250310003631
[Indexed for MEDLINE]

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