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Age-Related EBV-Associated B-Cell Lymphoproliferative Disorders Constitute a Distinct Clinicopathologic Group: A Study of 96 Patients

Authors' Affiliations: Departments of ¹ Clinical Oncology, ² Hematology and Cell Therapy, ³ Pathology and Molecular Diagnostics, and ⁴ Division of Molecular Medicine, Aichi Cancer Center, ⁵ Department of Hematology, Nagoya University Graduate School of Medicine, and ⁶ Department of Pathology and Clinical Laboratories, Nagoya University Hospital, Nagoya, Japan; ⁷ Department of Pathology, The Cancer Institute of the Japanese Foundation for Cancer Research, and ⁸ Department of Pathology, Teikyo University School of Medicine, Tokyo, Japan; ⁹ Department of Pathology, Saitama Cancer Center, Saitama, Japan; ¹⁰ Department of Pathology, School of Medicine, Kurume University, Kurume, Japan; ¹¹ First Department of Internal Medicine, Fukuoka University School of Medicine, Fukuoka, Japan; ¹² First Department of Pathology, Fukushima Medical College, Fukushima, Japan; and ¹³ Department of Pathology, Okayama University Graduate School of Medicine and Dentistry, Okayama, Japan

Requests for reprints: Kazuhito Yamamoto, Department of Hematology and Cell Therapy, Aichi Cancer Center, 1-1 Kanokoden, Chikusa-ku, Nagoya 464-8681, Japan. Phone: 81-52-762-6111; Fax: 81-52-764-2941; E-mail: kyamamoto{at}aichi-cc.jp.

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.