Age-related epstein-barr virus (EBV) associated B-cell lymphoproliferative disorders: Comparison with EBV-positive classic 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, Shigeo Nakamura

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Abstract

Age-related Epstein-Barr virus-associated IB-cell lymphoproliferative disorder (aEBVLPD) is a disease group characterized by EBV- associated large B-cell lymphoma in the elderly without predisposing immunodeficiency. In nearly one-third of cases, aEBV- LPD 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, aEBV-LPD had a significantly poorer prognosis than EBV+ cHL (P < .001). The polymorphous subtype of a EBVLPD constitutes an aggressive group with an immune response distinct from EBV+ cHL, and requires the development of innovative therapeutic strategies.

Original languageEnglish
Pages (from-to)2629-2636
Number of pages8
JournalBlood
Volume113
Issue number12
DOIs
Publication statusPublished - Mar 19 2009

ASJC Scopus subject areas

  • Biochemistry
  • Immunology
  • Hematology
  • Cell Biology

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    Asano, N., Yamamoto, K., Tamaru, J. I., Oyama, T., Ishida, F., Ohshima, K., Yoshino, T., Nakamura, N., Mori, S., Yoshie, O., Shimoyama, Y., Morishima, Y., Kinoshita, T., & Nakamura, S. (2009). Age-related epstein-barr virus (EBV) associated B-cell lymphoproliferative disorders: Comparison with EBV-positive classic hodgkin lymphoma in elderly patients. Blood, 113(12), 2629-2636. https://doi.org/10.1182/blood-2008-06-164806