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01-04-2016 | Original Article

Intrafollicular Epstein-Barr virus-positive large B cell lymphoma. A variant of “germinotropic” lymphoproliferative disorder

Authors: Luisa Lorenzi, Silvia Lonardi, Murad H. M. Essatari, Vilma Pellegrini, Simona Fisogni, Anna Gazzola, Claudio Agostinelli, William Vermi, Giuseppe Rossi, Giovannino Massarelli, Stefano A. Pileri, Fabio Facchetti

Published in: Virchows Archiv | Issue 4/2016

Abstract

Germinotropic lymphoproliferative disorders were previously described as localized disorders associated with coinfection by human herpes virus 8 and Epstein-Barr virus and characterized by good clinical outcome. We report the clinical, morphological, phenotypical, and molecular features of three cases of a hitherto unreported variant of Epstein-Barr virus (EBV)-positive, human herpes virus 8 (HHV8)-negative large B cell lymphoma with exclusive intrafollicular localization. All cases occurred in elderly individuals (63, 77, and 65 years old; one male, two females) without obvious immunedeficiency, who presented with high stage disease. Lymph nodes showed an effaced nodular architecture with abnormal B follicles colonized by EBV+ large, pleomorphic atypical cells, including Reed-Sternberg-like cells, showing an activated B cell phenotype (CD10-FOXP1-Bcl6-IRF4+ or CD10-FOXP1+Bcl6+IRF4+) and intense expression of CD30. No monoclonal light-chain restriction was detected by immunohistochemistry or in situ hybridization, and IGH rearrangement was polyclonal; notably, EBV clonality was detectable in one case. Lymphoma cells in all cases showed diffuse expression of the c-Myc protein, while Bcl2 was dim or negative; moreover, the strong expression of phosphorylated-STAT3 in tumor cell nuclei suggested activation of the JAK-STAT pathway. FISH analysis was performed in two cases and showed no translocations of BCL2, BCL6, MYC, and PAX5 genes. Response to treatment was poor in 2/3 patients: one died after 18 months, one is alive with disease after 12 months. The intrafollicular EBV-positive large B cell lymphoma expands the spectrum of EBV-associated lymphoproliferative disorders in immunocompetent individuals.

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Title: Intrafollicular Epstein-Barr virus-positive large B cell lymphoma. A variant of “germinotropic” lymphoproliferative disorder
Authors: Luisa Lorenzi
Silvia Lonardi
Murad H. M. Essatari
Vilma Pellegrini
Simona Fisogni
Anna Gazzola
Claudio Agostinelli
William Vermi
Giuseppe Rossi
Giovannino Massarelli
Stefano A. Pileri
Fabio Facchetti
Publication date: 01-04-2016
Publisher: Springer Berlin Heidelberg
Published in: Virchows Archiv / Issue 4/2016
Print ISSN: 0945-6317
Electronic ISSN: 1432-2307
DOI: https://doi.org/10.1007/s00428-015-1902-9

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

Intrafollicular Epstein-Barr virus-positive large B cell lymphoma. A variant of “germinotropic” lymphoproliferative disorder

Abstract

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

Abstract

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