Desorden linfoproliferativo germinotrópico asociado al HHV8/KSVH y EBV

Abstract

We described a case of KSHV and EBV associated germinotropic lymphoproliferative disorder, the first report of this entity was made in Blood in 2002 (1), at the moment there are around 20 cases reported (2,3). Is classified as one of the HHV8/KSVH associated lymphoproliferative disorders (4)(5). Clinically is presented by immunocompetent patients like an indolent, localized adenopathy and showed favorable response to chemotherapy, radiotherapy, or surgery (6). The histology presents plasmablasts that are positive for both HHV8/KSVH and EBV, these plasmablasts are involving the germinal centers of the lymphoid follicles (6), microdissected KSHV-positive plasmablast shows a policlonal or oligoclonal pattern, this feature is the reason why this is a “disorder” and non a neoplasia (6).  Lymphomagenesis is produced by co-infection of germinal center B cells; the HHV8/KSVH stays in a latency state in the infected cell, then occurs the “second genetic hit” (7): Epstein Barr virus infection, in an immunocompetent host produce a germinotropic lymphoprolifetive disorder.