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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.
Submitted June 23, 2008
Department of Pathology and Clinical Laboratories, Nagoya University Hospital, 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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