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Chronic Active Epstein-Barr Virus Disease

Hiroshi Kimura1, Jeffrey I Cohen2

  • 1Department of Virology, Nagoya University Graduate School of Medicine, Nagoya, Japan.

Frontiers in Immunology
|January 30, 2018
PubMed

Insights

Chronic active Epstein-Barr virus (CAEBV) disease is a rare, progressive disorder characterized by uncontrolled EBV infection, leading to immunodeficiency and severe complications. Hematopoietic stem cell transplantation is the only effective treatment, while research explores underlying immune defects and genetic causes.

Area of Science:

  • Immunology
  • Virology
  • Hematology

Background:

  • Chronic active Epstein-Barr virus (CAEBV) disease is a rare and severe disorder.
  • It involves uncontrolled Epstein-Barr virus (EBV) infection, leading to high EBV DNA levels and organ infiltration by EBV-positive lymphocytes.
  • Clinical manifestations include fever, lymphadenopathy, splenomegaly, EBV hepatitis, and pancytopenia.

Purpose of the Study:

  • To summarize the characteristics, clinical presentation, and treatment of Chronic active Epstein-Barr virus (CAEBV) disease.
  • To highlight the progressive nature of the disease, including immunodeficiency and potential fatal outcomes like opportunistic infections, hemophagocytosis, multiorgan failure, and EBV-positive lymphomas.
  • To differentiate disease presentation in the US versus Asia and emphasize current research directions.

Main Methods:

  • This is a summary and review of existing knowledge on CAEBV.
  • Information is synthesized from clinical observations and research findings.
  • Focus is on disease characteristics, patient presentation, and treatment efficacy.

Main Results:

  • CAEBV is a progressive disease with elevated EBV DNA and lymphocyte infiltration.
  • Patients face risks of severe complications including opportunistic infections, hemophagocytosis, multiorgan failure, and lymphomas.
  • Disease presentation varies geographically, with US cases often involving B or T cells, and Asian cases involving T or NK cells.

Conclusions:

  • Hematopoietic stem cell transplantation is the only proven effective treatment for CAEBV.
  • Ongoing research aims to identify the causes of CAEBV, focusing on immune defects and genetic abnormalities.
  • Early diagnosis and effective treatment are crucial for managing this rare and life-threatening disorder.

Keywords:
DDX3XEpstein–Barr virus lymphomachronic active Epstein–Barr virushemophagocytosisinfectious mononucleosis

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