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Blood, 19 March 2009, Vol. 113, No. 12, pp. 2629-2636.
Prepublished online as a Blood First Edition Paper on December 15, 2008; DOI 10.1182/blood-2008-06-164806.


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Submitted June 23, 2008
Accepted November 14, 2008

Age-related EBV-associated B-cell lymphoproliferative disorders: comparison with EBV-positive classical Hodgkin lymphoma in elderly patients

Naoko Asano*, Kazuhito Yamamoto, Jun-Ichi Tamaru, Takashi Oyama, Fumihiro Ishida, Koichi Ohshima, Tadashi Yoshino, Naoya Nakamura, Shigeo Mori, Osamu Yoshie, Yoshie Shimoyama, Yasuo Morishima, Tomohiro Kinoshita, and Shigeo Nakamura

Department of Pathology and Clinical Laboratories, Nagoya University Hospital, Nagoya, Japan
Department of Hematology and Cell Therapy, Aichi Cancer Center, Nagoya, Japan
Department of Pathology, Saitama Medical Center, Saitama Medical School, Kawagoe, Japan
Department of Hematology, Nagoya 2nd Red Cross Hospital, Nagoya, Japan
Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto, Japan
Department of Pathology, School of Medicine, Kurume University, Kurume, Japan
Department of Pathology, Okayama University Graduate School of Medicine and Dentistry, Okayama, Japan
Department of Pathology, Tokai University School of Medicine, Kanagawa, Japan
Department of Pathology, Teikyo University School of Medicine, Tokyo, Japan
Department of Microbiology, Kinki University School of Medicine, Osaka, Japan
Department of Hematology, Nagoya University School of Medicine, Nagoya, Japan

* Corresponding author; email: naasano{at}shinshu-u.ac.jp.

Age-related Epstein-Barr virus-associated B-cell lymphoproliferative disorder (aEBVLPD) is a disease group characterized by EBV-associated large B-cell lymphoma in elderly adults without predisposing immunodeficiency. In nearly one-third of cases, aEBVLPD occurs as a polymorphous subtype with reactive cell-rich components, bearing a morphological similarity to classical Hodgkin lymphoma (cHL). The aim of this study was to clarify clinicopathological 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 further histopathologically characterized by a higher ratio of geographical 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, aEBVLPD had a significantly poorer prognosis than EBV+cHL (P<0.0001). The polymorphous subtype of aEBVLPD constitutes an aggressive group with an immune response distinct from EBV+cHL, and requires the development of innovative therapeutic strategies.


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J. I. Cohen, H. Kimura, S. Nakamura, Y.-H. Ko, and E. S. Jaffe
Epstein-Barr virus-associated lymphoproliferative disease in non-immunocompromised hosts: a status report and summary of an international meeting, 8-9 September 2008
Ann. Onc., September 1, 2009; 20(9): 1472 - 1482.
[Abstract] [Full Text] [PDF]



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