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

• DOI: 10.1182/blood.v99.7.2331
• Publication Date: 2002-10-11T18:33:52Z
• Authors: Eric Oksenhendler; Emmanuelle Boulanger; Lionel Galicier; Ming-Qing Du; Nicolas Dupin; Tim C. Diss; Rifat Hamoudi; Marie-Thérèse D...; Félix Agbalika; Chris Boshoff; Jean-Pierre Clauvel; Peter G. Isaacson; Véronique Meignin
• Keywords: Gene Rearrangement; Time Factors; Castleman Disease; Lymphoma, Non-Hodgkin; HIV; Immunoglobulins; HIV Infections; Antiviral Agents; 3. Good health; Cohort Studies; 03 medical and health sciences; 0302 clinical medicine; Herpesvirus 8, Human; Humans; RNA, Viral; Sarcoma, Kaposi; Follow-Up Studies; Neoplasm Staging

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 6 plasmablastic lymphoma/leukemia (3/3 KSHV/HHV8+ EBV−). The NHL incidence observed in this cohort study (101/1000 patient-years) is about 15-fold what is expected in the general HIV+ population. MCD-associated KSHV/HHV8+ NHL 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.