Clinical Features and Treatment Strategies of Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis

Crit Rev Oncol Hematol. 2002 Dec;44(3):259-72. doi: 10.1016/s1040-8428(02)00117-8.

Abstract

Epstein-Barr virus (EBV) is the major triggering factor producing hemophagocytic syndrome or hemophagocytic lymphohistiocytosis (HLH). In this review, diagnostic problems, clinical and histopathological features, and treatment strategies of EBV-HLH have been described. In patients with EBV-HLH, the EBV-infected T cells or natural killer (NK) cells are mostly mono- or oligo-clonally proliferating, where hypercytokinemia plays a major role and causes hemophagocytosis, cellular damage and dysfunction of various organs. Although the majority of EBV-HLH cases develop in apparently immunocompetent children and adolescents, it also occurs in association with infectious mononucleosis, chronic active EBV infection, familial HLH, X-linked lymphoproliferative disease, lymphoproliferative disease like peripheral T-cell lymphoma and NK cell leukemia. In terms of treatment, special therapeutic measures are required to control the cytokine storm generated by EBV and to suppress proliferating EBV-genome-containing cells, because the clinical courses are often fulminant and result in a poor outcome.

Publication types

  • Review

MeSH terms

  • Antiviral Agents / therapeutic use
  • Combined Modality Therapy
  • Cytokines / metabolism
  • Epstein-Barr Virus Infections / complications
  • Epstein-Barr Virus Infections / diagnosis*
  • Epstein-Barr Virus Infections / therapy*
  • Hematopoietic Stem Cell Transplantation
  • Histiocytosis, Non-Langerhans-Cell / diagnosis*
  • Histiocytosis, Non-Langerhans-Cell / etiology
  • Histiocytosis, Non-Langerhans-Cell / therapy*
  • Humans
  • Immunotherapy / methods
  • Killer Cells, Natural / virology
  • T-Lymphocytes / virology

Substances

  • Antiviral Agents
  • Cytokines