TY - JOUR
T1 - Pathobiology of Epstein-Barr virus-driven peripheral T-cell lymphomas
AU - Piccaluga, Pier Paolo
AU - Gazzola, Anna
AU - Agostinelli, Claudio
AU - Bacci, Francesco
AU - Sabattini, Elena
AU - Pileri, Stefano A.
PY - 2011/8
Y1 - 2011/8
N2 - 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-κB pathway and platelet-derived growth factor receptor inhibition may represent 2 options to be tested in clinical trials.
AB - 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-κB pathway and platelet-derived growth factor receptor inhibition may represent 2 options to be tested in clinical trials.
KW - EBV
KW - Natural killer
KW - Peripheral T-cell lymphomas
KW - T/NK lymphoproliferative disorder
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U2 - 10.1053/j.semdp.2011.02.007
DO - 10.1053/j.semdp.2011.02.007
M3 - Article
C2 - 21850988
AN - SCOPUS:79960112753
VL - 28
SP - 234
EP - 244
JO - Seminars in Diagnostic Pathology
JF - Seminars in Diagnostic Pathology
SN - 0740-2570
IS - 3
ER -