title: Primary effusion lymphoma presenting as a cutaneous intravascular lymphoma.
abstract: Primary effusion lymphoma (PEL) is a rare and aggressive lymphoma that arises in the context of immunosuppression and is characterized by co-infection with Epstein-Barr virus (EBV) and human herpesvirus-8/Kaposi sarcoma-associated herpesvirus (HHV-8/KSHV). It was originally described as arising in body cavity effusions, but presentation as a mass lesion (extracavitary PEL) is now recognized. Here, we describe a case of PEL with an initial presentation as an intravascular lymphoma with associated skin lesions. The patient was a 53-year-old man with human immunodeficiency virus (HIV)/acquired immune deficiency syndrome (AIDS) who presented with fevers, weight loss and skin lesions concerning for Kaposi sarcoma (KS). A skin biopsy revealed no evidence of KS; however, dermal vessels contained large atypical cells that expressed CD31 and plasma cell markers but lacked most B- and T-cell antigens. The atypical cells expressed EBV and HHV-8. The patient subsequently developed a malignant pleural effusion containing the same neoplastic cell population. The findings in this case highlight the potential for unusual intravascular presentations of PEL in the skin as well as the importance of pursuing microscopic diagnosis of skin lesions in immunosuppressed patients.
authors.affiliation: Department of Pathology, The Johns Hopkins Medical Institutions, Baltimore, MD, USA.
authors.forename: Genevieve M
authors.initials: GM
authors.lastname: Crane
authors.forename: Rena R
authors.initials: RR
authors.lastname: Xian
authors.forename: Kathleen H
authors.initials: KH
authors.lastname: Burns
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Borowitz
authors.forename: Amy S
authors.initials: AS
authors.lastname: Duffield
authors.forename: Janis M
authors.initials: JM
authors.lastname: Taube
mesh_terms: D001706:Biopsy; D003937:Diagnosis, Differential; D020031:Epstein-Barr Virus Infections / Q000175:diagnosis / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D006566:Herpesviridae Infections / Q000175:diagnosis / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D004854:Herpesvirus 4, Human / Q000302:isolation & purification; D019288:Herpesvirus 8, Human / Q000302:isolation & purification; D006801:Humans; D016483:Lymphoma, AIDS-Related / Q000175:diagnosis* / Q000378:metabolism / Q000473:pathology / Q000821:virology; D054685:Lymphoma, Primary Effusion / Q000175:diagnosis* / Q000378:metabolism / Q000473:pathology / Q000821:virology; D008297:Male; D008875:Middle Aged; D019408:Platelet Endothelial Cell Adhesion Molecule-1 / Q000378:metabolism; D016066:Pleural Effusion, Malignant / Q000175:diagnosis / Q000378:metabolism / Q000473:pathology / Q000821:virology; D012878:Skin Neoplasms / Q000175:diagnosis* / Q000378:metabolism / Q000473:pathology / Q000821:virology
chemical_list: D019408:Platelet Endothelial Cell Adhesion Molecule-1
keywords: Epstein-Barr virus (EBV); human herpesvirus type-8 (HHV-8); intravascular lymphoma; primary effusion lymphoma; skin biopsy