Age related Epstein Barr virus associated B cell lymphoproliferative disorder in Peru

Abstract

19514 Background: Age related Epstein Barr virus (EBV) associated B cell lymphoproliferative disorder is a recent new entity described in Japanese patients with diffuse large B cell lymphoma (DLBCL) and infection with EBV. It usually affects elderly patients with poor survival. Methods: In order to establish the correlation of EBV with DLBCL, we investigated the EBV status by RNA- in situ hybridization (ISH) in newly diagnosed nodal DLBCL.Between January 2002 and December 2004, seventy-four newly diagnosed nodal DLBCL patients were included in the analysis. Clinical data were reviewed retrospectively and patient’s biopsies were analyzed for the presence of EBV encoded RNA (EBER) by ISH and the immunohistochemical expression of BCL6, CD10 and MUM-1/IRF4 by tissue microarray (TMA) technique. Results: In this cohort the mean age was 66.1 years old (range 23–84) at diagnostic; male/female ratio 39/35; 46 patients (65.7%) with advanced stage (III/IV); 53 cases (71.6%) were of the non Germinal Center DLBCL type (non GC). This group was associated with a higher IPI than the Germinal Center DLBCL type (GC). Also there was a trend that the non GC type had a poorer overall survival than the GC type (p=0.171). Eleven cases of the cohort (14.9%) were identified as EBER-positive and were called as Age related EBV associated B cell lymphoproliferative disorder. Most of the cases EBER (+) were of the non GC group (10/11), showing a trend between EBER+ and non-GC DLBCL (OR=0.21, p=0.11 fisher 2-tails). EBER positive cases were associated with an advanced age (> 60 years), poorer status performance, more advanced stage, higher IPI risk group and poorer outcome to initial treatment. Overall survival in the EBER positive cases and negative were 1.1 vs. 15.5 months respectively (p=0.001). At the multivariable level de EBER status is an independent variable compared with the International Prognostic Index (IPI) (p=0.001) with a 3.2-fold (95% CI, 1.5 - 7.2) risk for death for positive cases Conclusions: Age related EBV associated B cell lymphoproliferative disorder is frequent between DLBCL in Peru. It was related to more advanced age, poor status performance, non-GC group and very short overall survival in comparison with DLBCL. New treatment strategies should be defined in this subgroup of poor prognostic. No significant financial relationships to disclose.