Clinicopathologic and molecular biological study of senile/age-related EBV-associated B-cell

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Malignant lymphoma is widely heterogeneous in its clinical, biologic, and endemic properties. Epstein-Barr virus (EBV) is associated with some disease entities of malignant lymphomas, including Burkitt lymphoma, Hodgkin lymphoma, immunodeficiency-associated lymphoproliferative disorders (LPDs) and a part of diffuse large B-cell lymphoma. In 2003, we reported 22 cases named as senile EBV-associated B-cell lymphoproliferative disorders (B-LPDs), which were divided into polymorphic and large-cell lymphoma (LCL) subtypes, arising in elderly patients aged >60 years without predisposing immunodeficiencies (Am J Surg Pathol 2003;27:16). Tumor cells expressed CD20 and/or CD79a, and in situ hybridization showed them to be associated with EBV. LMP1 was detected in all cases and EBNA2 in seven (32%), indicating latency II and III status respectively. This disease was often morphologically accompanid by a varying number of HRS-like giant cells, and suggested to have a relationship with an immunolo … More gical deterioration derived from the aging process.In order to further address the clinicopathologic profiles and spectrum for this newly-recognized disease entity, we subsequently searched 134 cases of the EBV-positive B-cell LPDs without underlying immunodeficiency-related diseases during the period from January 1990 and December 2003 as the multi-institutional study, which have been retrieved from 2,000 patients with diffuse large B-cell lymphoma and the related diseases. Their overall clinicopthologic profiles are now under investigation in details. In brief, this larger series revealed that 126 (94%) of the patients are more than 50 years old of age, providing an additional evidence that EBV-positive LPDs without predisposing immunodeficiency mainly occurs in elderly patients. However, 8 patients were found to be less than 50 years old of age, although it remains to be adequately elucidated about their relationship with chronic active EBV infection and/or primary immune disorders. In taking these rare cases into the consideration, the term of "age-related" may be more appropriate than that of "senile" for further understanding the overall age distribution of EBV-positive B-cell LPDs without predisposing immunodeficiency. Less