Short report

Treatment of EBV driven lymphoproliferation with erythrophagocytosis: 12 year follow up

C A Bethune¹, M M Gompels³, C Taylor⁴, B Angus², G P Spickett¹

¹ Department of Immunology, Royal Victoria Infirmary, Newcastle Upon Tyne NE1 4LP, UK
² Department of Histopathology, Royal Victoria Infirmary
³ Department of Immunology, Royal Victoria Infirmary, Newcastle Upon Tyne NE1 4LP, UK
⁴ Department of Virology, Public Health Laboratory, Newcastle General Hospital, Newcastle upon Tyne NE4 6BE, UK

Correspondence to:
Dr Bethune

This is a report of a case of Epstein-Barr virus (EBV) associated haemophagocytic syndrome in a 17 year old woman with antibody deficiency. For two years before this presentation, serology showed abnormally high titres to EBV early antigen, suggestive of persistent infection with EBV. She became acutely unwell with clinical features consistent with virus associated haemophagocytic syndrome (VAHS). Histology showed lymphoproliferation with erythrophagocytosis and evidence of EBV encoded RNAs in liver, spleen, and lymph node. VAHS is often fatal, particularly when it occurs in patients with underlying immunodeficiencies. In this case, treatment with intravenous immunoglobulin, aciclovir, and interferon was followed by a dramatic recovery. Twelve years later the patient remains relatively well on regular intravenous immunoglobulin.

Key Words: Epstein-Barr virus associated haemophagocytic syndrome • antibody deficiency • interferon

CiteULike    Complore    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this?

This article has been cited by other articles:

• Casper, C., Nichols, W. G., Huang, M.-L., Corey, L., Wald, A. (2004). Remission of HHV-8 and HIV-associated multicentric Castleman disease with ganciclovir treatment. Blood 103: 1632-1634 [Abstract] [Full Text]  
• van Diest, P J, Holzel, H (2002). Cervical cancer. J. Clin. Pathol. 55: 241-242 [Full Text]