Treatment of Epstein-Barr virus-related hemophagocytic lymphohistiocytosis (EBV-HLH); update 2010

J Pediatr Hematol Oncol. 2011 Jan;33(1):35-9. doi: 10.1097/MPH.0b013e3181f84a52.

Abstract

The clinical features of Epstein-Barr virus-related hemophagocytic lymphohistiocytosis (EBV-HLH) vary significantly, from mild to severe, at the time of the treatment decision. There are many reports of successful treatments, for example conservative treatment without etoposide, HLH-94/2004-type immunochemotherapy with etoposide, and hematopoietic stem cell transplantation. When considering the treatment of EBV-HLH, the most important factor is the finding that a survival benefit is obtained when etoposide-containing therapy is initiated within 4 weeks of diagnosis. This indicates that there may be a window for observation or conservative corticosteroid/cyclosporine A or intravenous immunoglobulin (IVIG) treatment; however, once the disease is defined as "high risk" and/or refractory to such therapy, prompt introduction of etoposide (ideally within 4 wk) is recommended. In deciding whether the disease is "high-risk," evaluation of clinical staging, EBV genome copy numbers in the serum, cellular EBV tropism, chromosome analysis, and screening for hereditary immuno-deficient diseases such as familial HLH, are required.

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Antiviral Agents / therapeutic use
  • Cyclosporine / therapeutic use
  • Epstein-Barr Virus Infections / diagnosis
  • Epstein-Barr Virus Infections / genetics
  • Epstein-Barr Virus Infections / therapy*
  • Etoposide / therapeutic use
  • Hematopoietic Stem Cell Transplantation
  • Humans
  • Immunoglobulins / therapeutic use
  • Lymphohistiocytosis, Hemophagocytic / diagnosis
  • Lymphohistiocytosis, Hemophagocytic / therapy*
  • Neoplasm Staging
  • Risk Factors
  • Treatment Outcome

Substances

  • Adrenal Cortex Hormones
  • Antiviral Agents
  • Immunoglobulins
  • Etoposide
  • Cyclosporine