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Chronic active Epstein-Barr virus infection (CAEBV) is one of the Epstein-Barr virus (EBV)-positive T- or NK-lymphoproliferative diseases. It is considered rare and geographically limited to Japan and East Asia. However, CAEBV is drawing international attention, and the number of case reported worldwide is increasing, after its classification in the EBV-positive T- or NK-cell neoplasms, in the 2016 WHO classification. In this article, I review current advances in the study of CAEBV under the new definition and show future directions. In CAEBV, EBV-infected T or NK cells clonally proliferate and infiltrate multiple organs, leading to their failure. These characteristics define CAEBV as a lymphoid neoplasm. However, the main symptom of CAEBV is inflammation. Recently, the mechanisms underlying the development of CAEBV have gradually become clearer. EBV infection of T or NK cells can occur during the acute phase of primary infection with a high EBV load in the peripheral blood. In addition, it was reported that cytotoxic T cells decreased in numbers or showed dysfunction in CAEBV. These findings suggest that undetermined immunosuppressive disorders may underlie persistent infection of T or NK cells. Furthermore, EBV itself contributes to the survival of host cells.  In vitro  EBV infection of T cells induced intercellular survival-promoting pathways. Constitutive activation of NF-kB and STAT3 was observed in EBV-positive T or NK cells in CAEBV, promoting not only cell survival but also CAEBV development. During the disease course, CAEBV can lead to two lethal conditions: hemophagocytic lymphohistiocytosis and chemotherapy-resistant lymphoma. It is necessary to start treatment before these conditions develop. At present, the only effective treatment strategy for eradicating EBV-infected T or NK cells is allogeneic stem cell transplantation (allo-HSCT). However, patients with an active disease, in which the condition is accompanied by fever, liver dysfunction, progressive skin lesions, vasculitis, or uveitis, had worse outcomes after allo-HSCT, than patients with an inactive disease had. Unfortunately, current chemotherapies are insufficient to improve the activity of CAEBV. Based on the molecular mechanisms for the development of the disease, the NF-kB, or JAK/STAT mediating pathways are attractive candidate targets for new treatments.

frontiers in pediatrics

Advances in the Study of Chronic Active Epstein-Barr Virus Infection: Clinical Features Under the 2016 WHO Classification and Mechanisms of Development