KSHV/HHV8 associated B-cell lymphoid proliferations and lymphomas

WHO 2022 recognizes the full spectrum of lymphoid proliferations related to Kaposi sarcoma herpesvirus/human herpesvirus 8 (KSHV/HHV8). These lymphoid proliferations include

• KSHV/HHV8-associated multicentric Castleman disease (KSHV/HHV8-MCD)

• germinotropic lymphoproliferative disorder (KSHV/HHV8-GLPD)

• primary effusion lymphoma (PEL)

• extracavitary PEL (EC-PEL)

• KSHV/HHV8-positive diffuse large B-cell lymphoma (KSHV/ HHV8-DLBCL)

PEL/EC-PEL and KSHV/HHV8-DLBCL are characteristically seen in HIV patients, but can be seen in other immunodeficiency settings. KSHV/HHV8-GLPD, in contrast, is more prevalent in elderly patients without overt immunodeficiency, although it has also been reported in HIV-positive individuals. In addition, KSHV/HHV8-MCD is seen in both HIV-positive and HIVnegative patients, but the latter are overall older.