Background: Anaplastic lymphoma kinase-positive diffuse large B-cell lymphoma (ALK-DLBCL) is a rare lymphoma with several clinicopathological differences from ALK-positive anaplastic large cell lymphoma (ALCL). The latest WHO classification of lymphomas recognizes ALK-DLBCL as a separate entity.
EBV-positive diffuse large B-cell lymphoma (DLBCL) of the elderly is an entity recently included in the WHO classification of lymphoid tumors. We have reviewed our experience and clinical outcomes of this distinct subtype of DLBCL. Between 2002 and 2009, cases of DLBCL were identified from medical records of the Hospital Nacional Edgardo Rebagliati Martins in Lima, Peru, and underwent pathological evaluation including immunohistochemistry for CD20, CD10, bcl-6, MUM1/IRF4, and EBV-encoded RNA in situ hybridization. Clinical data were gathered, tabulated, and reported descriptively. Survival analyses were performed using Kaplan-Meier estimates. Out of 199 cases of DLBCL, 28 cases of EBV-positive DLBCL of the elderly were identified. The median age was 75 years with male predominance (1.5:1). B-symptoms were present in 43%, advanced stage in 50% and International Prognostic Index (IPI) score > 2 in 57% of patients; 68% of patients had a nongerminal center (NGC) phenotype. The complete response rates to R-CHOP and CHOP were 63% and 33%, respectively. The median overall survival (OS) for the group was 5 months. In the univariate analysis, age 70 years, lymphocyte count <1.0 3 10 9 /L, and advanced clinical stage were associated with worse OS in patients treated with chemotherapy with and without rituximab. EBV-positive DLBCL of the elderly is a clinically aggressive entity with a short OS and typically presents with advanced stage, high IPI score, and a NGC phenotype. Further studies are needed to investigate if rituximab-containing regimens are associated with better response and OS rates in EBV-positive DLBCL of the elderly. Am. J. Hematol. 86:663-667, 2011. V
Although the International Prognostic Index (IPI) score is a valuable prognostic tool in diffuse large B-cell lymphoma (DLBCL), other risk-stratifying factors may be of value. The aim of this study was to define the prognostic value of EBV expression in de novo nodal DLBCL. Seventy-four cases were selected between January 2002 and December 2007. Clinical data were reviewed and tissue samples were evaluated for expression of CD20, CD10, bcl-6, MUM1, and EBV-encoded RNA (EBER). Of 74 evaluated cases, 53 cases (72%) were of non-germinal center-like subtype and 11 cases (15%) were positive for EBER. In a univariate analysis of the 57 patients who received chemotherapy, factors associated with survival were EBV status, performance status, LDH level, and IPI score. Using a multivariate analysis, a prognostic model was developed using IPI score and EBV status, which showed statistical significance. Our study supports EBV status as a powerful prognostic factor in de novo nodal DLBCL. Prospective studies should be carried to validate this hypothesis.
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