Human herpesvirus 8-related primary effusion lymphoma in four HIV-uninfected patients without organ transplantation

Primary effusion lymphoma (PEL) is a rare subtype of non-Hodgkin's lymphoma. PEL is closely related in pathogenesis to human herpesvirus 8 (HHV-8) and typically occurs in patients with significant immunodeficiency. Cases of PEL in patients without either HIV infection or transplantation have been reported from HHV-8-endemic areas, but very rarely from the Asia-Pacific region. In this case series, we describe the clinical and immunohistochemical presentations of four patients in southern Taiwan who were diagnosed with HHV-8-related PEL. All four patients were HIV-negative and had not received organ transplantation. To our knowledge, this is the first authentic report of HIV-unrelated PEL from the ethnically Chinese population. Considering the non-specific clinical manifestations, the pivotal roles of specific assays for correct diagnosis, and the dismal prognosis of PEL, it is important for clinicians to include PEL as one of the differential diagnosis when approaching HIV-uninfected patients with unexplained exudative body cavity effusion.