TY - JOUR
T1 - Age-related epstein-barr virus (EBV) associated B-cell lymphoproliferative disorders
T2 - Comparison with EBV-positive classic hodgkin lymphoma in elderly patients
AU - Asano, Naoko
AU - Yamamoto, Kazuhito
AU - Tamaru, Jun Ichi
AU - Oyama, Takashi
AU - Ishida, Fumihiro
AU - Ohshima, Koichi
AU - Yoshino, Tadashi
AU - Nakamura, Naoya
AU - Mori, Shigeo
AU - Yoshie, Osamu
AU - Shimoyama, Yoshie
AU - Morishima, Yasuo
AU - Kinoshita, Tomohiro
AU - Nakamura, Shigeo
PY - 2009/3/19
Y1 - 2009/3/19
N2 - Age-related Epstein-Barr virus-associated IB-cell lymphoproliferative disorder (aEBVLPD) is a disease group characterized by EBV- associated large B-cell lymphoma in the elderly without predisposing immunodeficiency. In nearly one-third of cases, aEBV- LPD occurs as a polymorphous subtype with reactive cell-rich components, bearing a morphologic similarity to classic Hodgkin lymphoma (cHL). The aim of this study was to clarify clinicopathologic differences between the polymorphic subtype of aEBVLPD (n = 34) and EBV + cHL (n = 108) in patients aged 50 years or older. Results showed that aEBVLPD was more closely associated with aggressive clinical parameters than cHL, with a higher age at onset (71 vs 63 years); lower male predominance (male-female ratio, 1.4 vs 3.3); and a higher rate of involvement of the skin (18% vs 2%), gastrointestinal tract (15% vs 4%), and lung (12% vs 2%). aEBVLPD was histopathologically characterized by a higher ratio of geographic necrosis, greater increase (> 30%) in cytotoxic T cells among background lymphocytes, higher positivity for CD20 and EBNA2, and absence of CD15 expression. As predicted by the clinical profile, aEBV-LPD had a significantly poorer prognosis than EBV+ cHL (P < .001). The polymorphous subtype of a EBVLPD constitutes an aggressive group with an immune response distinct from EBV+ cHL, and requires the development of innovative therapeutic strategies.
AB - Age-related Epstein-Barr virus-associated IB-cell lymphoproliferative disorder (aEBVLPD) is a disease group characterized by EBV- associated large B-cell lymphoma in the elderly without predisposing immunodeficiency. In nearly one-third of cases, aEBV- LPD occurs as a polymorphous subtype with reactive cell-rich components, bearing a morphologic similarity to classic Hodgkin lymphoma (cHL). The aim of this study was to clarify clinicopathologic differences between the polymorphic subtype of aEBVLPD (n = 34) and EBV + cHL (n = 108) in patients aged 50 years or older. Results showed that aEBVLPD was more closely associated with aggressive clinical parameters than cHL, with a higher age at onset (71 vs 63 years); lower male predominance (male-female ratio, 1.4 vs 3.3); and a higher rate of involvement of the skin (18% vs 2%), gastrointestinal tract (15% vs 4%), and lung (12% vs 2%). aEBVLPD was histopathologically characterized by a higher ratio of geographic necrosis, greater increase (> 30%) in cytotoxic T cells among background lymphocytes, higher positivity for CD20 and EBNA2, and absence of CD15 expression. As predicted by the clinical profile, aEBV-LPD had a significantly poorer prognosis than EBV+ cHL (P < .001). The polymorphous subtype of a EBVLPD constitutes an aggressive group with an immune response distinct from EBV+ cHL, and requires the development of innovative therapeutic strategies.
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U2 - 10.1182/blood-2008-06-164806
DO - 10.1182/blood-2008-06-164806
M3 - Article
C2 - 19075188
AN - SCOPUS:63849290800
VL - 113
SP - 2629
EP - 2636
JO - Blood
JF - Blood
SN - 0006-4971
IS - 12
ER -