A Unique Case of Malignant Pleuropericardial Effusion : HHV-8-Unrelated PEL-Like Lymphoma—A Case Report and Review of the Literature

Abstract EN

Primary effusion lymphoma (PEL) or body cavity lymphoma is a rare type of extra nodal lymphoma of B-cell origin that presents as lymphomatous effusion(s) without any nodal enlargement or tumor masses.

It belongs to the group of AIDS related non-Hodgkin’s lymphomas.

First described in 1996 in HIV infected individuals who were coinfected with Kaposi’s sarcoma-associated herpesvirus (KSHV) or HHV-8 virus, it was included as a separate entity in WHO classification of tumors of hematopoietic and lymphoid tissue in the year 2001.

The definition included association with HHV-8 virus as a mandatory diagnostic criterion.

However, cases were later reported where PEL-like disease process was diagnosed in HHV-8 negative patients.

This was eventually recognized as a rare but distinct entity termed as “HHV-8-unrelated PEL-like lymphoma”.

Herein, we are reporting a case of an elderly patient who presented with a large pleuropericardial effusion and was eventually diagnosed with this entity.

Till date, only around 50 cases of HHV-8-unrelated PEL-like lymphoma have been reported and our case being EBV, HIV, and Hepatitis C negative makes it very unique and rare occurrence.

We are also presenting a review of relevant literature focused mainly on comparing outcomes in patients treated with and without chemotherapy.