title: KSHV/HHV8-negative effusion-based lymphoma, a distinct entity associated with fluid overload states.
abstract: Human herpesvirus-8 (HHV8)-positive effusion-based lymphomas have been termed primary effusion lymphoma (PEL) in the WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. Kaposi sarcoma herpesvirus (KSHV)/HHV8-negative effusion-based lymphomas (KSHV/HHV8-negative EBLs) resembling PELs have been reported in the literature and in many cases have been (mis)classified as PEL-like lymphomas. Herein, we present a series of cases and a review of KSHV/HHV8-negative EBLs. This lymphoma, although cytomorphologically resembling PEL, is a distinct entity with characteristic clinical and pathologic features. Patients are older, generally human immunodeficiency virus negative and not immunosuppressed, frequently hepatitis C positive compared with the population baseline, and often have an underlying medical condition leading to fluid overload. The lymphoma cells express pan-B-cell antigens in 86.7%, and CD20 is expressed in 71.1% of the cases. The lymphoma is often of germinal center B or mixed germinal center B/activated B-cell signature with the Hans classifier, and Epstein-Barr virus is positive in nearly 30% of cases. Rare T-cell lymphomas were also reported. Clinical outcomes and response to therapy, including isolated aspiration, are relatively favorable compared with cases of PEL. We suggest that HHV8-negative effusion-based lymphoma is a distinct entity associated with fluid overload states.
authors.affiliation: David Geffen School of Medicine at UCLA, CA, USA.
authors.forename: Serge
authors.initials: S
authors.lastname: Alexanian
authors.forename: Jonathan
authors.initials: J
authors.lastname: Said
authors.forename: Mark
authors.initials: M
authors.lastname: Lones
authors.forename: Sheeja T
authors.initials: ST
authors.lastname: Pullarkat
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D018951:Antigens, CD20 / Q000378:metabolism; D014408:Biomarkers, Tumor / Q000378:metabolism; D017809:Fatal Outcome; D005260:Female; D019288:Herpesvirus 8, Human / Q000302:isolation & purification*; D006801:Humans; D054685:Lymphoma, Primary Effusion / Q000150:complications / Q000378:metabolism / Q000473:pathology* / Q000628:therapy; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012514:Sarcoma, Kaposi / Q000150:complications / Q000378:metabolism / Q000473:pathology*; D016896:Treatment Outcome; D014883:Water-Electrolyte Imbalance / Q000150:complications / Q000378:metabolism / Q000473:pathology*
chemical_list: D018951:Antigens, CD20; D014408:Biomarkers, Tumor