High incidence of Kaposi sarcoma-associated herpesvirus-related non-Hodgkin lymphoma in patients with HIV infection and multicentric Castleman disease

Multicentric Castleman disease (MCD) is a distinct type of lymphoproliferative disorder associated with inflammatory symptoms and interleukin 6 (IL-6) dysregulation. In the context of human immunodeficiency virus (HIV) infection, MCD is associated with Kaposi sarcoma-associated herpesvirus, also called human herpesvirus type 8 (KSHV/HHV8). Within a prospective cohort study on 60 HIV-infected patients with MCD, and a median follow-up period of 20 months, 14 patients developed KSHV/HHV8-associated non-Hodgkin lymphoma (NHL): 3 "classic" KSHV/HHV8(+) Epstein-Barr virus-positive (EBV+) primary effusion lymphoma (PEL), 5 KSHV/HHV8(+) EBV(-)visceral large cell NHL with a PEL-like phenotype, and 13 plasmablastic lymphoma/leukemia (3/3 KSHV/HHV8(+) EBV-). The NHL incidence observed in this cohort study (101/1000 patient-years) is about 15-old:what is expected in the general HIV+ population. MCD-assoclated KSHV/HHV8(+) NFIL fell into 2 groups, suggesting different pathogenesis. The plasmablastic NHL likely represents the expansion of plasmablastic microlymphoma, from the MCD lesion and progression toward aggressive NHL. In contrast, the PEL and PEL-like NHL may implicate a different original infected cell whose growth is promoted by the cytokine-rich environment of the MCD lesions. (C) 2002 by The American Society of Hematology.