Lymphoproliferative Disorders with Concurrent HHV8 and EBV Infection: Beyond Primary Effusion Lymphoma and Germinotropic Lymphoproliferative Disorder

Abstract

Aims

Lymphoproliferative disorders (LPD) characterized by human herpesvirus 8 (HHV8) and Epstein-Barr virus (EBV) infection are rare and two entities are included in the World and Health Organization classification: primary effusion lymphoma (PEL) and germinotropic LPD. These two entities have very different clinicopathological presentations and prognosis. Here we describe two cases of HHV8-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 localized 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 centers penetrated by sclerotic blood vessels and surrounded by concentric rings of small lymphocytes. By immunohistochemistry, the lymphoma cells were positive for CD3 (weak), CD20, and HHV8. EBV was positive as shown by in situ hybridization. The lymphoma cells were negative for CD138, IgM, 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 HHV8 and EBV, partially positive for CD20 and CD138, and negative for kappa and lambda.

Conclusions

The description of these two cases suggests that the category of HHV8+ EBV+ LPDs is a spectrum, which not only includes PEL and germinotropic LPD, but also other cases that have overlapping but distinctive features.

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