Primary Effusion Lymphoma

Epidemiology

§   Universally associated with HHV-8/ Kaposi sarcoma herpes virus (mediterrannean)

§   Often occurs in the presence of immunodeficiency

Clinical Features

§   Effusions in the absence of lymphadenopathy/ organomegaly

§   Some have pre-existing KS

§   Rarely associated with mulitcentric Castlemans

Aetiology

§   Neoplastic cells positive for HHV8/ KSHV

§   Most cases coinfected with EBV

Morphology

§   Large pleomorphic cells

Immunophenotype

§   CD45 positive

§   Negative for pan B markers

§   May express PC related markers – CD30, CD38, CD138

§   Express HHV8/KSHV

Prognosis

§   poor