title: Pathobiology of Epstein-Barr virus-driven peripheral T-cell lymphomas.
abstract: In the present review, the authors described the pathobiological features of Epstein-Barr virus (EBV)-driven T/natural killer cell-derived malignancies. These rare tumors appear to be quite heterogeneous with regard to both clinical and pathologic features. Nonetheless, some elements, especially regarding the possible role of EBV (ie, genomic predisposition, pathogenesis, pattern of latency), are similar, enforcing the concept of a causative role for the virus. In clinical practice, although definitely rare in Western countries, the tumors are not exceptional; thus, they should be taken into account in the differential diagnosis of T-lymphoproliferative disorders, also considering the need for extremely prompt intervention. The prognosis of such tumors is generally poor using current approaches. A better understanding of their molecular pathogenesis may lead to significant therapeutic improvements. For example, the nuclear factor-KB pathway and platelet-derived growth factor receptor inhibition may represent 2 options to be tested in clinical trials.
authors.affiliation: Molecular Pathology Laboratory, Hematopathology Section, Department of Hematology and Oncological Sciences L. and A. Seràgnoli, S. Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy. pierpaolo.piccaluga@unibo.it
authors.forename: Pier Paolo
authors.initials: PP
authors.lastname: Piccaluga
authors.forename: Anna
authors.initials: A
authors.lastname: Gazzola
authors.forename: Claudio
authors.initials: C
authors.lastname: Agostinelli
authors.forename: Francesco
authors.initials: F
authors.lastname: Bacci
authors.forename: Elena
authors.initials: E
authors.lastname: Sabattini
authors.forename: Stefano A
authors.initials: SA
authors.lastname: Pileri
mesh_terms: D002471:Cell Transformation, Neoplastic / Q000473:pathology; D020031:Epstein-Barr Virus Infections / Q000150:complications* / Q000473:pathology; D006801:Humans; D054391:Lymphoma, Extranodal NK-T-Cell / Q000473:pathology / Q000821:virology*; D016411:Lymphoma, T-Cell, Peripheral / Q000473:pathology / Q000821:virology*