Age-related Epstein-Barr virus (EBV)–associated B-cell lymphoproliferative disorders: comparison with EBV-positive classic Hodgkin lymphoma in elderly patients

Blood - Tập 113 Số 12 - Trang 2629-2636 - 2009
Naoko Asano1,2,3, Kazuhito Yamamoto4, Jun‐ichi Tamaru5, Takashi Oyama6, Fumihiro Ishida7, Koichi Ohshima8, Tadashi Yoshino9, Naoya Nakamura10, Shigeo Mori11, Osamu Yoshie12, Yoshie Shimoyama2, Yasuo Morishima4, Tomohiro Kinoshita13, Shigeo Nakamura2
1Department of Laboratory Medicine, Shinshu University School of Medicine, Matsumoto
2Department of Pathology and Clinical Laboratories, Nagoya University Hospital, Nagoya;
3Department of Pathology and Molecular Diagnostics, Aichi Cancer Center, Nagoya
4Department of Hematology and Cell Therapy, Aichi Cancer Center, Nagoya;
5Department of Pathology, Saitama Medical Center, Saitama Medical School, Kawagoe;
6Department of Hematology, Nagoya 2nd Red Cross Hospital, Nagoya;
7Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto
8Department of Pathology, School of Medicine, Kurume University, Kurume;
9Department of Pathology, Okayama University Graduate School of Medicine and Dentistry, Okayama.
10Department of Pathology, Tokai University School of Medicine, Isehara
11Department of Pathology, Teikyo University School of Medicine, Tokyo;
12Department of Microbiology, Kinki University School of Medicine, Osaka; and
13Department of Hematology, Nagoya University Graduate School of Medicine, Nagoya, Japan

Tóm tắt

Abstract 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 without predisposing immunodeficiency. In nearly one- third of cases, aEBVLPD 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, aEBVLPD had a significantly poorer prognosis than EBV+ cHL (P < .001). 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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Blood

Tập 113 Số 12

2629-2636

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