Treatment of EBV driven lymphoproliferation with erythrophagocytosis: 12 year follow up
- 1Department of Immunology, Royal Victoria Infirmary, Newcastle Upon Tyne NE1 4LP, UK
- 2Department of Histopathology, Royal Victoria Infirmary
- 3Department of Immunology, Royal Victoria Infirmary, Newcastle Upon Tyne NE1 4LP, UK
- 4Department of Virology, Public Health Laboratory, Newcastle General Hospital, Newcastle upon Tyne NE4 6BE, UK
- Dr Bethune
- Accepted 13 June 2000
Abstract
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 α interferon was followed by a dramatic recovery. Twelve years later the patient remains relatively well on regular intravenous immunoglobulin.
