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Lymphoproliferative disorders with concurrent HHV 8 and EBV infection: beyond primary effusion lymphoma and germinotropic lymphoproliferative disorder
Author(s) -
Wang Wei,
KanagalShamanna Rashmi,
Medeiros L Jeffrey
Publication year - 2018
Publication title -
histopathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.626
H-Index - 124
eISSN - 1365-2559
pISSN - 0309-0167
DOI - 10.1111/his.13428
Subject(s) - primary effusion lymphoma , lymphoma , medicine , pathology , lymphoproliferative disorders , hodgkin's lymphoma , germinal center , immunohistochemistry , epstein–barr virus , biopsy , mantle zone , antibody , virus , immunology , b cell
Aims Lymphoproliferative disorders ( LPD ) characterised by human herpesvirus 8 ( HHV 8) and Epstein–Barr virus ( EBV ) infection are rare and two entities are included in the World Health Organisation classification: primary effusion lymphoma ( PEL ) and germinotropic LPD . These two entities have very different clinicopathological presentations and prognosis. Here we describe two cases of HHV 8‐positive, EBV ‐positive lymphoma with clinicopathological features that are not consistent with either PEL or germinotropic LPD . Methods and results Both patients were HIV ‐negative without a history of immunodeficiency. In the first case, the patient presented with localised axillary lymphadenopathy without any other symptoms. Biopsy showed lymphoma cells located predominantly in mantle zones with extension into interfollicular areas. Some follicles showed Castleman features, including lymphocyte‐depleted germinal centres penetrated by sclerotic blood vessels and surrounded by concentric rings of small lymphocytes. Using immunohistochemistry, the lymphoma cells were positive for CD 3 (weak), CD 20 and HHV 8. EBV was positive, as shown by in‐situ hybridisation. The lymphoma cells were negative for CD 138, immunoglobulin (Ig)M and kappa and lambda. In the second case, the patient presented with multi‐compartmental lymphadenopathy and biopsy showed neoplastic cells that grew in a diffuse pattern with immunoblastic/plasmablastic morphology. Neoplastic cells were positive for both HHV 8 and EBV , partially positive for CD 20 and CD 138 and negative for kappa and lambda. Conclusions The description of these two cases suggests that the category of HHV 8 + EBV + LPD s is a spectrum, which not only includes PEL and germinotropic LPD , but also other cases that have overlapping but distinctive features.