BACKGROUND
Primary effusion lymphoma (PEL) is a rare and distinct type of
high-grade non-Hodgkin’s B-cell lymphoma (NHL) [1]. Human
herpesvirus type 8 (HHV8) been directly linked to PEL since the late
1990s [2].
PEL is characterized by the presence of significant serous lymphomatous
fluid collection in the pleural, pericardial, and peritoneal cavities
without detectable solid tumor masses [3].
It most often occurs in immunocompromised patients such as in the
advanced stages of Acquired Immune Deficiency Syndrome (AIDS) [4].
PEL has also been reported in immunocompetent elderly patients from
HHV-8 endemic areas such as central and southern Africa and the
Mediterranean area. It typically involves Middle-aged patients with a
significant male predominance of 6:1 [5].
The clinical scenario is directly related to the body cavity involved,
the amount of the effusion, and its interaction with the adjacent
structures [6].
The diagnosis of PEL is based on cytological immunophenotypically and
viral characteristics of the involved effusion. PEL is suspected in the
presence of diffuse large cells with abundant basophilic cytoplasm and
irregular nuclei with prominent nucleoli resembling plasma cells
[7].
The lymphoma cells usually express the leukocyte origin marker CD45 but
lack the typical B or T cell phenotype while markers of plasma cell
differentiation, CD38, and CD138 are usually present [8].
Uniform treatment of PEL has not been properly established, and patients
are generally treated with combined chemotherapy used for aggressive
lymphomas known as the ”CHOP” protocol, consisting of
cyclophosphamide, hydroxydaunorubicin, oncovin (vincristine) and
prednisone. Despite complete response rates of 43 to 57%, the median
survival is still poor and estimated as six to nine months, due to early
and usually refractory relapses [9].