Kaposi's sarcoma-associated herpesvirus/human herpesvirus 8 associated lymphoproliferative disorders

Eric Oksenhendler, David Boutboul and Lionel Galicier


Kaposi's sarcoma-associated herpesvirus/Human herpesvirus 8 (KSHV/HHV-8) is associated with multicentric Castleman disease (MCD) and primary effusion lymphoma (PEL), two unique lymphoproliferative disorders (LPDs). In MCD, KSHV/HHV-8-infected B cells, although polyclonal, express a monotypic IgMλ phenotype, probably through revision of the B cell receptor and editing towards lambda light chain in mature B cells. Despite a phenotype of plasmablasts, they do not harbor somatic mutations and are considered to originate from pre-germinal center (GC) naive B cells. Both viral and human IL-6 play a role in the plasmacytic differentiation and growth of these cells. In addition, viral replication can be observed in some KSHV/HHV-8-infected cells. PEL lymphomatous cells are clonal B cells harboring numerous somatic mutations and usually considered as GC/post-GC B cells. One can also hypothesize that they originate from the same infected naive B cells and that additional factors, including EBV co-infection, could be responsible for their peculiar phenotype.

  • Submitted November 14, 2018.
  • Accepted December 27, 2018.