Age-related EBV-associated B-cell lymphoproliferative disorders constitute a distinct clinicopathologic group: a study of 96 patients.
OBJECTIVE
We have recently reported EBV+ B-cell lymphoproliferative disorders (LPD) occurring predominantly in elderly patients, which shared features of EBV+ B-cell neoplasms arising in the immunologically deteriorated patients despite no predisposing immunodeficiency and were named as senile or age-related EBV+ B-cell LPDs. To further characterize this disease, age-related EBV+ B-cell LPDs were compared with EBV-negative diffuse large B-cell lymphomas (DLBCL).


METHODS
Among 1,792 large B-cell LPD cases, 96 EBV+ cases with available clinical data set were enrolled for the present study. For the control group, 107 patients aged over 40 years with EBV-negative DLBCL were selected. We compared clinicopathologic data between two groups and determined prognostic factors by univariate and multivariate analysis.


RESULTS
Patients with age-related EBV+ B-cell LPDs showed a higher age distribution and aggressive clinical features or parameters than EBV-negative DLBCLs: 44% with performance status >1, 58% with serum lactate dehydrogenase level higher than normal, 49% with B symptoms, and higher involvement of skin and lung. Overall survival was thus significantly inferior in age-related EBV+ group than in DLBCLs. Univariate and multivariate analyses further identified two factors, B symptoms and age older than 70 years, independently predictive for survival. A prognostic model using these two variables well defined three risk groups: low risk (no adverse factors), intermediate risk (one factor), and high risk (two factors).


CONCLUSIONS
These findings suggest that age-related EBV+ B-cell LPDs constitute a distinct group, and innovative therapeutic strategies such as EBV-targeted T-cell therapy should be developed for this uncommon disease.
Oyama
Takashi
T
Department of Clinical Oncology, Nagoya University Graduate School of Medicine, Nagoya, Japan.
Yamamoto
Kazuhito
K
Asano
Naoko
N
Oshiro
Aya
A
Suzuki
Ritsuro
R
Kagami
Yoshitoyo
Y
Morishima
Yasuo
Y
Takeuchi
Kengo
K
Izumo
Toshiyuki
T
Mori
Shigeo
S
Ohshima
Koichi
K
Suzumiya
Junji
J
Nakamura
Naoya
N
Abe
Masafumi
M
Ichimura
Koichi
K
Sato
Yumiko
Y
Yoshino
Tadashi
T
Naoe
Tomoki
T
Shimoyama
Yoshie
Y
Kamiya
Yoshikazu
Y
Kinoshita
Tomohiro
T
Nakamura
Shigeo
S
D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D001402:B-Lymphocytes / Q000473:pathology*; D015496:CD4-Positive T-Lymphocytes / Q000276:immunology; D005260:Female; D004854:Herpesvirus 4, Human / Q000302:isolation & purification*; D006689:Hodgkin Disease / Q000473:pathology; D006801:Humans; D016130:Immunophenotyping; D016393:Lymphoma, B-Cell / Q000473:pathology; D016403:Lymphoma, Large B-Cell, Diffuse / Q000473:pathology; D008232:Lymphoproliferative Disorders / Q000188:drug therapy / Q000401:mortality / Q000473:pathology* / Q000821:virology; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D011379:Prognosis; D012189:Retrospective Studies