EBV-associated lymphoproliferative disorders/lymphomas
Authors: Shigeo Nakamura
Journal: Annals of Oncology (2015)

Background: EBV is well known to be associated with several lymphoproliferative disorder (LPD) and/or lymphoma, e.g., Burkitt lymphoma, nasal type natural killer (NK)/T-cell lymphoma, classical Hodgkin lymphoma, and immunodeficiency-associated LPDs. In these days, novel EBV+ LPDs/lymphomas are increasingly recognized in non-immunocompromised hosts, both in Asia and Western countries. They include T/NK cell type chronic active EBV infection (CAEBV) and age-related EBV+ B-cell LPDs, which are now listed in the 2008WHO classification as systemic EBV+ T-cell LPD of childhood and EBV+ diffuse large B-cell lymphoma (DLBCL) of the elderly, respectively. They generally have a broad clinicopathologic spectrum spanning from the precursor lesion to overt lymphoma, often posing the differential diagnostic problems to the pathologists. In addition, we are now facing the newly standing-up diseases.Design: The EBV+ LPDs/lymphomas were reviewed for our further understanding their clinicopathologic features.Results: Age-related EBV+ B-LPD consisted of some subgroups, EBV+ hyperplasia, mucocuntaneous ulcer, polymorphous LPD and DLBCL, and constitutes a continuous spectrum with EBV+ classical Hodgkin lymphoma. Some are associated with iatrogenic immunodeficiency besides aging and show spontaneous regression in the clinical course. CAEBV type LPD is a systemic disease with a clonal proliferation of T and NK cells in peripheral blood, and is related with clinical variants, i.e., EBV-associated hemophagocytic syndrome, severe mosquito bite allergy and hydroa vacciniforme. Nodal EBV+ cytotoxic T-cell lymphoma also has just been documented as the distinct disease from nasal type lymphoma.Conclusion: These EBV+ LPDs/lymphomas always request us to refine the disease definitions and concepts more accurately and to innovate our therapeutic approaches. Background: EBV is well known to be associated with several lymphoproliferative disorder (LPD) and/or lymphoma, e.g., Burkitt lymphoma, nasal type natural killer (NK)/T-cell lymphoma, classical Hodgkin lymphoma, and immunodeficiency-associated LPDs. In these days, novel EBV+ LPDs/lymphomas are increasingly recognized in non-immunocompromised hosts, both in Asia and Western countries. They include T/NK cell type chronic active EBV infection (CAEBV) and age-related EBV+ B-cell LPDs, which are now listed in the 2008WHO classification as systemic EBV+ T-cell LPD of childhood and EBV+ diffuse large B-cell lymphoma (DLBCL) of the elderly, respectively. They generally have a broad clinicopathologic spectrum spanning from the precursor lesion to overt lymphoma, often posing the differential diagnostic problems to the pathologists. In addition, we are now facing the newly standing-up diseases. Design: The EBV+ LPDs/lymphomas were reviewed for our further understanding their clinicopathologic features. Results: Age-related EBV+ B-LPD consisted of some subgroups, EBV+ hyperplasia, mucocuntaneous ulcer, polymorphous LPD and DLBCL, and constitutes a continuous spectrum with EBV+ classical Hodgkin lymphoma. Some are associated with iatrogenic immunodeficiency besides aging and show spontaneous regression in the clinical course. CAEBV type LPD is a systemic disease with a clonal proliferation of T and NK cells in peripheral blood, and is related with clinical variants, i.e., EBV-associated hemophagocytic syndrome, severe mosquito bite allergy and hydroa vacciniforme. Nodal EBV+ cytotoxic T-cell lymphoma also has just been documented as the distinct disease from nasal type lymphoma. Conclusion: These EBV+ LPDs/lymphomas always request us to refine the disease definitions and concepts more accurately and to innovate our therapeutic approaches.