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

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

Catherine M. Bollard1-3 and Jeffrey I. Cohen4
1Center for Cancer and Immunology Research, Children’s National Health System, Washington, DC; 2Department of Pediatrics and 3Department of Microbiology,
Immunology and Tropical Medicine, The George Washington University, Washington, DC; and 4Laboratory of Infectious Diseases, National Institute of Allergy and
Infectious Diseases, National Institutes of Health, Bethesda, MD
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. (Blood. 2018;131(26):2899-2905)