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The Oncologist, Vol. 13, No. 5, 577-585, May 2008; doi:10.1634/theoncologist.2008-0036
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 EBV-Associated Lymphoproliferative Disorders: Classification and Treatment
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Lymphoma

EBV-Associated Lymphoproliferative Disorders: Classification and Treatment

Antonino Carbonea, Annunziata Gloghinia, Giampietro Dottib

aDepartment of Pathology, Fondazione IRCCS Istituto Nazionale Tumori, Milano, Italy; bCenter for Cell and Gene Therapy, Baylor College of Medicine, The Methodist Hospital, Houston, Texas, USA

Key Words. EBV • EBV-associated lymphomas • Post-transplant–associated lymphoproliferative disorders • HIV-associated lymphoproliferative disorders • EBV cell–based immunotherapy

Correspondence: A. Carbone, M.D., Department of Pathology, Fondazione IRCCS Istituto Nazionale Tumori, via Venezian 1, Milano I-20133, Italy. Telephone: 39-02-2390-2876; Fax: 39-02-2390-2877; e-mail: antonino.carbone{at}istitutotumori.mi.it

Received February 13, 2008; accepted for publication March 18, 2008.

Disclosure: No potential conflicts of interest were reported by the authors, planners, reviewers, or staff managers of this article.

Since its discovery as the first human tumor virus, Epstein-Barr virus (EBV) has been implicated in the development of a wide range of B-cell lymphoproliferative disorders, including Burkitt's lymphoma, classic Hodgkin's lymphoma, and lymphomas arising in immunocompromised individuals (post-transplant and HIV-associated lymphoproliferative disorders). T-cell lymphoproliferative disorders that have been reported to be EBV associated include a subset of peripheral T-cell lymphomas, angioimmunoblastic T-cell lymphoma, extranodal nasal type natural killer/T-cell lymphoma, and other rare histotypes. EBV encodes a series of products interacting with or exhibiting homology to a wide variety of antiapoptotic molecules, cytokines, and signal transducers, hence promoting EBV infection, immortalization, and transformation. However, the exact mechanism by which EBV promotes oncogenesis is an area of active debate. The focus of this review is on the pathology, diagnosis, classification, and pathogenesis of EBV-associated lymphomas. Recent advances in EBV cell–based immunotherapy, which is beginning to show promise in the treatment of EBV-related disorders, are discussed.






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