Age-related EBV-associated B-cell lymphoproliferative disorders constitute a distinct clinicopathologic group: a study of 96 patients

Clin Cancer Res. 2007 Sep 1;13(17):5124-32. doi: 10.1158/1078-0432.CCR-06-2823.


Purpose: 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).

Experimental design: 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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Age Factors
  • Aged
  • Aged, 80 and over
  • B-Lymphocytes / pathology*
  • CD4-Positive T-Lymphocytes / immunology
  • Female
  • Herpesvirus 4, Human / isolation & purification*
  • Hodgkin Disease / pathology
  • Humans
  • Immunophenotyping
  • Lymphoma, B-Cell / pathology
  • Lymphoma, Large B-Cell, Diffuse / pathology
  • Lymphoproliferative Disorders / drug therapy
  • Lymphoproliferative Disorders / mortality
  • Lymphoproliferative Disorders / pathology*
  • Lymphoproliferative Disorders / virology
  • Male
  • Middle Aged
  • Multivariate Analysis
  • Prognosis
  • Retrospective Studies