INTRODUCTION
Primary effusion lymphoma (PEL) is a rare aggressive B-cell neoplasm
that presents with serous malignant effusions without detectable tumor
associated mass.1 It is related with HHV8 infection
and mainly affects immunocompromised patients such as HIV-infected
individuals, recipients of solid-organ transplantation or elderly
patients.3 Moreover, the coinfection with Epstein-Barr
virus (EBV) is frequent. The term “primary effusion lymphoma” was
first described in the literature in 1996 by Nador et
al.3 to define malignant lymphomatous effusions
related to HHV8 infection. In 2001, the World Health Organization (WHO)
introduced the category of PEL only for HHV8-related primary
lymphomatous effusions. In 1996 Hermine et al.4reported the first HHV8 negative lymphomatous effusion in an
HIV-seronegative patient. Since then, more than 60 HHV8-unrelated
effusion-based lymphomas (EBL) have been reported in the literature,
most of them in Japan, and terms as EBL and PEL-like lymphomas have been
used. Recently, the ICC of Mature Lymphoid Neoplasms has recognized
these cases as a new provisional entity named HHV8 and EBV-negative
primary effusion-based lymphoma.2 Moreover, WHO
Classification, 5th edition, has also recently recognized this entity as
fluid overload-associated large B-cell lymphoma.5
We report a 72-year-old patient that presented with pericardial effusion
with no evidence of tumor mass. Morphologic, immunophenotypic and
molecular studies of the tumor cells of the pericardial effusion were
performed. Cytological analysis showed large cells with irregular
nuclei, prominent nucleoli and vacuolized cytoplasm. Immunophenotype
showed expression of CD45, CD19, CD20 without detection of surface
immunoglobulin. The tumor cells were negative for LANA-1 and EBER.
Finally, PEL-like was diagnosed. To date, clinical and pathologic
features of HHV8-unrelated, effusion-based lymphomas (EBL) are poorly
understood. We report herein a case of the new entity HHV8- and
EBV-negative primary effusion-based lymphoma, presenting in a Caucasian
woman and review the literature.