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

Abstract

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.

Primary immune regulatory disorders for the pediatric hematologist and oncologist: A case‐based review

“…Three of these five received cytotoxic T‐cell therapies directed toward EBV post HSCT. Similarly, the existing literature describing outcomes of HSCT for this population documents an overall survival of approximately 70% . The optimal approach to decrease EBV burden in these patients prior to transplantation, as well as how to optimally condition for transplant, is currently unknown.…”

“…Treatment approaches have included immunomodulatory therapy, antivirals, replication inhibitors, and chemotherapy. Although these can control viral burden and tissue damage, only allogeneic transplant (from an EBV + donor) is considered curative …”

“…Similarly, the existing literature describing outcomes of HSCT for this population documents an overall survival of approximately 70%. 72,76,77 The optimal approach to decrease EBV burden in these patients prior to transplantation, as well as how to optimally condition for transplant, is currently unknown. Treatment approaches have included immunomodulatory therapy, antivirals, replication inhibitors, and chemotherapy.…”

“…Although these can control viral burden and tissue damage, only allogeneic transplant (from an EBV + donor) is considered curative. 76,77…”

A clinicopathologic study of the spectrum of systemic forms of EBV‐associated T‐cell lymphoproliferative disorders of childhood: A single tertiary care pediatric institution experience in North America

“…Based on patients’ clinical characteristics, laboratory information, disease courses, and pathologic and immunophenotypic findings, we attempted to classify cases according to classification previously proposed by Kimura et al and 2017 WHO classifications . Of note, the diagnostic criteria for S‐CAEBV are relatively more straightforward with current clinical and pathological criteria . However, the published guidelines for classification between EBV‐HLH and S‐EBV‐TCL are not clear, and the two terminologies are often used interchangeably by different authors .…”