HHV-8 associated lymphoma

The discovery of Kaposi sarcoma herpesvirus (KSHV) / human herpesvirus 8 (HHV-8) led to recognition of primary effusion lymphoma (PEL) as a distinct lymphoproliferative disorder. Subsequently, two other types of lymphoma have been shown to be associated with HHV-8 : HHV-8 positive diffuse large B cell lymphoma not otherwise specified and germinotropic lymphoproliferative disorder.The pathogenesis of PEL is unique as in most cases the HHV-8+ tumoral cells are coinfected with the Epstein-Barr virus (EBV), suggesting an interplay between these two herpesviruses. This article reviews advances in the field of characterization of the lymphomatous cells, pathogenesis, and targeted therapies.The gene expression profile analysis of PEL cells and the experimental coinfection of peripheral B cells with HHV-8 and EBV allow dissection of the mechanisms of lymphomagenesis and of the relative contribution of both viruses. Systemic chemotherapy regimen remains poorly effective but new therapeutic perspectives are open with the use of monoclonal antibodies, immunomodulatory drugs, and immunotherapy.HHV-8 associated lymphoma is a model for studying virus-induced lymphoproliferation and its relation with host immune response and PEL is a unique model to study the relative contribution of two herpesviruses to lymphomagenesis in coinfected cells.