How I treat T-cell chronic active Epstein-Barr virus disease

Blood. 2018 Jun 28;131(26):2899-2905. doi: 10.1182/blood-2018-03-785931. Epub 2018 Apr 30.


T-cell chronic active Epstein-Barr virus (CAEBV) is a rare disease in which EBV is present predominantly in T cells that infiltrate the tissues; patients have high levels of EBV in the blood. If untreated, patients often develop liver failure, hemophagocytic lymphohistiocytosis, coronary artery aneurysms, EBV infiltrating T cells impairing organ function, or T-cell lymphomas refractory to treatment. At present, hematopoietic stem-cell transplantation is the only curative therapy, and it is critical to make a proper diagnosis and initiate transplantation before the disease progresses to an irreversible stage. Specific medications such as high-dose systemic corticosteroids or ganciclovir combined with either histone deacetylase inhibitors or bortezomib may temporarily reduce systemic toxicity associated with T-cell CAEBV and allow the patient time to receive a transplant. Relapses of the disease after transplantation have also occurred, and the use of donor-derived virus-specific T cells may help to treat these relapses.

Publication types

  • Case Reports
  • Research Support, N.I.H., Intramural

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Adult
  • Antiviral Agents / therapeutic use
  • Child
  • Chronic Disease
  • Disease Management
  • Disease Progression
  • Epstein-Barr Virus Infections / complications
  • Epstein-Barr Virus Infections / diagnosis*
  • Epstein-Barr Virus Infections / pathology
  • Epstein-Barr Virus Infections / therapy*
  • Female
  • Ganciclovir / therapeutic use
  • Hematopoietic Stem Cell Transplantation* / methods
  • Herpesvirus 4, Human / drug effects
  • Herpesvirus 4, Human / isolation & purification*
  • Histone Deacetylase Inhibitors / therapeutic use
  • Humans
  • Male
  • T-Lymphocytes / pathology
  • T-Lymphocytes / virology*


  • Adrenal Cortex Hormones
  • Antiviral Agents
  • Histone Deacetylase Inhibitors
  • Ganciclovir