Abstract
Chronic active Epstein–Barr virus infection (CAEBV) is a disease where Epstein–Barr virus (EBV)-infected T- or NK-cells are activated and proliferate clonally. The symptoms of this dual-faced disease include systemic inflammation and multiple organ failures caused by the invasion of infected cells: inflammation and neoplasm. At present, the only effective treatment strategy to eradicate EBV-infected cells is allogeneic stem cell transplantation. Lately, the investigation into the disease’s pathogenic mechanism and pathophysiology has been advancing. In this review, I will evaluate the new definition in the 2017 WHO classification, present the advancements in the study of CAEBV, and unfold the future direction.
Highlights
It is known that Epstein–Barr virus (EBV) causes B-cell lymphomas such as Burkitt lymphoma and diffuse large B-cell lymphoma, but their genome is known to be positive in certain T- or NK-cell lymphoid neoplasms such as extranodal NK/T-cell lymphoma (ENKL), aggressive NK-cell leukemia (ANKL), and chronic active Epstein–Barr virus infection (CAEBV)
MHLW research group defines CAEBV as a disease characterized by infectious mononucleosis (IM)-like symptoms and systemic inflammation such as fever, lymphadenopathy, hepatosplenomegaly, an increased amount of EBV genome in peripheral blood (PB) or in lesion tissue, and EBV infection of T- or NK-cells
The three-year survival rate in the cases treated by hematopoietic stem cell transplant (HSCT) only was 82%, that of chemotherapy followed by HSCT was 65%, and that of chemotherapy only was a tough result of 0%
Summary
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