RESUMO
Chronic active EBV (CAEBV) infection presents with persistent or recurring IM-like symptoms including fever, hepatosplenomegaly, lymphadenopathy, and liver dysfunction without definite underlying diseases at the diagnosis. No causative pathogenetic mechanisms have been sufficiently clarified, these patients have high EBV-DNA load in the peripheral blood and systemic clonal expansion of EBV-infected T cells, NK cells and B cells. Their prognosis is poor with life-threatening complications including hemophagocytic lymphohistiocytosis, diverse organ failure, malignant lymphomas and so on. Allogeneic hematopoietic stem cell transplantation(Allo-HSCT)is the only effective therapy at present. This review outlines the recent progress of CAEBV clinical and fundamental research at home and abroad.