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

• Published in: Blood 2002-04, Vol.99 (7), p.2331-2336
• Main Authors: Oksenhendler, Eric, Boulanger, Emmanuelle, Galicier, Lionel, Du, Ming-Qing, Dupin, Nicolas, Diss, Tim C., Hamoudi, Rifat, Daniel, Marie-Thérèse, Agbalika, Félix, Boshoff, Chris, Clauvel, Jean-Pierre, Isaacson, Peter G., Meignin, Véronique
• Format: Article
• Language: English
• Subjects: Antiviral Agents - therapeutic use; Biological and medical sciences; Castleman Disease - complications; Castleman Disease - genetics; Castleman Disease - pathology; Cohort Studies; Follow-Up Studies; Gene Rearrangement; Hematologic and hematopoietic diseases; Herpesvirus 8, Human - genetics; Herpesvirus 8, Human - isolation & purification; HIV - isolation & purification; HIV Infections - complications; HIV Infections - drug therapy; HIV Infections - genetics; Human viral diseases; Humans; Immunoglobulins - genetics; Infectious diseases; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; Lymphoma, Non-Hodgkin - etiology; Lymphoma, Non-Hodgkin - genetics; Lymphoma, Non-Hodgkin - pathology; Lymphoma, Non-Hodgkin - virology; Medical sciences; Neoplasm Staging; RNA, Viral - isolation & purification; Sarcoma, Kaposi - complications; Sarcoma, Kaposi - drug therapy; Sarcoma, Kaposi - genetics; Sarcoma, Kaposi - pathology; Time Factors; Viral diseases; Viral diseases of the lymphoid tissue and the blood. Aids
• ISSN: 0006-4971; 1528-0020
• DOI: 10.1182/blood.V99.7.2331

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.