Abstract
Epstein-Barr virus-positive diffuse large B-cell lymphoma of the elderly (EBV+ DLBCL-e) is a molecularly distinct variant of DLBCL, characterized by a monoclonal B-cell proliferation that occurs in patients >50 years of age without a history or clinicopathologic evidence of immunodeficiency. However, patients with EBV+ DLBCL younger than 50-years-old also exist in Western countries. We evaluated the clinicopathologic, immunophenotypic and genetic features in Cacausian patients with EBV+ DLBCL who are ≤50 years of age and compared this patient group to patients who are >50 years. In patients who are ≤50 years, less frequent expression of BCL6 and a trend of more frequent expression of CD30 and pSTAT3 were found in patients with EBV+ DLBCL. In patients who are >50 years, common expression of CD30, p50, pSTAT3 and less frequent expression of BCL6 were observed. Older patients also more commonly had a poor performance status (ECOG≥2). Comparing EBV+ DLBCL patients in ≤50 years versus >50 years, both groups had similar clinicopathologic, immunophenotypic and genetic features. Gene expression profiling, microRNA profiling and treatment outcome of the younger patients with EBV+ DLBCL was not distinctive from tumors in older patients. Based on our data, we suggest that the arbitrary age cutoff for EBV+ DLBCL is unnecessary and should be eliminated in the WHO lymphoma classification scheme.
Highlights
Epstein-Barr virus (EBV) positive diffuse large B-cell lymphoma of the elderly (EBV+ DLBCL-e) is a monoclonal B-cell lymphoid proliferation that occurs in patients > 50 years without evidence of immunodeficiency or a history of lymphoma [1]
EBV+ DLBCL-e is a provisional entity in the current World Health Organization (WHO) classification
The notion that DLBCL associated with EBV could harbor a worse outcome has been generally accepted among pathologists and oncologists, but for the age cutoff of 50 years there has been some degree of resistance in the community
Summary
Epstein-Barr virus (EBV) positive diffuse large B-cell lymphoma of the elderly (EBV+ DLBCL-e) is a monoclonal B-cell lymphoid proliferation that occurs in patients > 50 years without evidence of immunodeficiency or a history of lymphoma [1]. EBV+ DLBCL-e constitutes 8-10% and 2-5% of DLBCL in Asian countries and Western countries, respectively [2,3,4,5]. In the era of treatment with cyclophosphamide, doxorubicin, vincristine and prednisone (CHOP), patients with EBV+ DLBCL-e were thought to be an aggressive variant of DLBCL. With the current therapeutic regimen, CHOP plus rituximab (R-CHOP), EBV+DLBCL does not confer a worse prognosis in Western patients [5, 7]. The data is controversial in Asian patients [8, 9]
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