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

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 interferon was followed by a dramatic recovery. Twelve years later the patient remains relatively well on regular intravenous immunoglobulin. (J Clin Pathol 2001;54:328-331)

Remission of HHV-8 and HIV-associated multicentric Castleman disease with ganciclovir treatment

“…Antivirals may help prevent the development of EBV-related B-cell lymphoproliferative disorders that are characterized by latent EBV and polyclonal tumors 23 but have little role in their treatment, a situation analogous to KS. 24 Conversely, the response to acyclovir has been documented in fulminant infectious mononucleosis 25 and the HPS 26,27 in which replication of EBV may play a larger role in the disease process, perhaps analogous to the role of HHV-8 in MCD. For personal use only.…”

Treatment of Epstein‐Barr virus‐associated hemophagocytic lymphohistiocytosis (EBV‐HLH) in young adults: A report from the HLH studyl center

“…There are reports that plasmapheresis [12,17,18], intravenous immunoglobulin (IVIG) [19,20], cyclosporin A (CSA) [21,22], etoposide [3,7,12], and a combination of IVIG and etoposide [23] have been effective. However, there is no consensus in the literature, which of these treatments is optimal [24].…”

Combining an antiviral with rituximab in EBV-related haemophagocytic lymphohistiocytosis led to rapid viral clearance; and a comprehensive review

“…For instance, a combination of acyclovir (750 mg/day), methylprednisolone (1 g/day for 3 days), IVIG (20 g/day for 3 days) and gabexate (an antitumour necrosis factor agent, 2 g/day) administered in an effort to avoid the toxicity of chemotherapy, induced remission in one case of a pregnant woman 32. Similarly, the combination of IVIG, α interferon and steroids was successful in two patients 33 34. In another case, the addition of gancyclovir to high EBV-titre IVIG and cyclosporine resulted in clinical and VL remission after no clinical or VL response to etoposide.…”