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Blood, 1 April 2002, Vol. 99, No. 7, pp. 2331-2336

CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS

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

Eric Oksenhendler, Emmanuelle Boulanger, Lionel Galicier, Ming-Qing Du, Nicolas Dupin, Tim C. Diss, Rifat Hamoudi, Marie-Thérèse Daniel, Félix Agbalika, Chris Boshoff, Jean-Pierre Clauvel, Peter G. Isaacson, and Véronique Meignin

From the Department of Immunology and Hematology, Laboratory of Hematology, Laboratory of Virology, CRC Viral Oncology Group, Department of Pathology, Hôpital Saint-Louis and the Department of Dermatology, NADER, Hôpital Tarnier-Cochin, Paris, France; the Department of Histopathology, Royal Free and University College Medical School, and The Wolfson Institute for Biomedical Research, University College London, London, United Kingdom.

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.

© 2002 by The American Society of Hematology.
 

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