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Case Reports in Hematology
Volume 2017 (2017), Article ID 1578429, 8 pages
https://doi.org/10.1155/2017/1578429
Case Report

HHV8/EBV Coinfection Lymphoproliferative Disorder: Rare Entity with a Favorable Outcome

1Pathology Department, Mohamed Tahar Maamouri Hospital, Mrezga, Nabeul, Tunisia
2Medicine Faculty of Tunis, Tunis El Manar University, Tunis, Tunisia

Correspondence should be addressed to Dhouha Bacha; moc.liamg@ahcabahuohd

Received 30 November 2016; Accepted 10 January 2017; Published 9 February 2017

Academic Editor: German Pihan

Copyright © 2017 Dhouha Bacha et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

HHV8/EBV-associated germinotropic lymphoproliferative disorder (GLD) is a challenging diagnosis given its rarity, the particular clinical presentation, and the lack of expression of markers usually used in establishing hematopoietic lineage. We report a new case of HHV8/EBV GLD in an immunocompetent 78-year-old woman. The diagnosis was made in an incidentally discovered lymphadenopathy. Histological examination showed a nodular lymphoid proliferation centered by aggregates of atypical plasmablastic cells admixed with small lymphoid cells. Tumor cells were strongly positive with EMA, HHV8, LMP1, CD38, CD138, and kappa light chains. They were negative with common lymphoma-associated markers (CD20, CD3, CD15, CD30, CD10, and bcl2). In situ hybridization confirmed the monotypic kappa light chains and the EBV infection (EBER+). A polyclonal pattern of Ig gene rearrangement was detected by PCR analysis. In the adjacent lymph node parenchyma, some germinal centers mimicked Castleman disease. In this case, the differential diagnosis was discussed with an early stage of large B-cell lymphoma arising in HHV8-associated multicentric Castleman disease. The clinical presentation, the immunophenotype, and the molecular results helped to make the accurate diagnosis. Through the review of the nine previously reported cases in literature, we discuss the clinical and pathologic features and the differential diagnosis of HHV8/EBV GLD.