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.
Keywords: EBV-positive T-cell lymphoma of childhood; WHO classification; cutaneous chronic active Epstein–Barr virus infection; systemic chronic active Epstein–Barr virus infection.