| Summary: | Epstein Barr virus (EBV) is associated with a wide range of human lymphoproliferative disorders (LPD) of B, T, and natural killer (NK)-cell lineage. In children, abnormal immune response to primary EBV infection can cause peculiar forms of T/NK- cell LPD of childhood, such as the systemic form of chronic active EBV infection, hydroa vacciniforme-like LPD, severe mosquito bite allergy and systemic T cell lymphoma of childhood. In adults, dysregulation of the immune response to EBV infection, immunosenescence caused by aging, chronic inflammation in a closed space, and iatrogenic immune suppression can lead to EBV-positive LPD of diverse types involving B, T, and NK cells with unique clinical and pathological presentations. This review describes the clinical, pathological, and genetic findings of EBV-positive LPD listed in the revised 2016 WHO Classification of Tumours of Haematopoietic and Lymphoid Tissue.
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