Primary Pleural Lymphoma
Body cavity-based lymphoma; PAL; PEL; PPL; Primary effusion lymphoma; Pyothorax-associated lymphoma
Secondary involvement of the pleura by systemic lymphoma is more common than primary lymphomas arising in the pleura. It is generally accepted that two distinct forms of primary pleural lymphoma (PPL) exist: primary effusion lymphoma (PEL) and pyothorax-associated lymphoma (PAL).
Primary effusion lymphoma (PEL), also termed body cavity-based lymphoma, is a large B-cell non-Hodgkin lymphoma that often presents with serous effusions and no detectable mass lesions and carries strong associations with human herpes virus 8(HHV8)/Kaposi sarcoma herpes virus (KSHV) and human immunodeficiency virus (HIV). Immunodeficiency is a hallmark association of PEL, regardless of the underlying etiology of the immunodeficiency, though a majority of cases arise specifically in association with HIV infection (Patel and Xiao 2013). However, cases have been reported in HIV-negative...