title: HHV8-related lymphoid proliferations: a broad spectrum of lesions from reactive lymphoid hyperplasia to overt lymphoma.
abstract: Human herpesvirus 8 (HHV8)-associated lymphoid proliferations are uncommon and poorly characterized disorders mainly affecting immunosuppressed patients, especially with HIV infection. They encompass different diseases with overlapping features that complicate their classification. In addition, the role of HHV8 in reactive lymphoid hyperplasia is not well known. To analyze the clinicopathological spectrum of these lesions, we have reviewed 66 biopsies of 61 patients with HHV8 infection. All cases were also investigated for Epstein-Barr virus (EBV) and HIV infection. We identified 13 (20%) cases of HHV8-related reactive lymphoid hyperplasia, 2 (3%) HHV8 plasmablastic proliferations of the splenic red pulp, 28 (42%) multicentric Castleman disease, 6 (9%) germinotropic lymphoproliferative disorders, and 17 (26%) HHV8-related lymphomas. As expected, the pathologic subtype was predictive of overall survival (P<0.05). Forty-seven of our cases were HIV positive (77%). In addition to the classical presentation of the different entities, we identified novel and overlapping features. Reactive HHV8 proliferations were frequently associated with systemic symptoms but never progressed to overt HHV8-positive lymphoma. Two cases had a plasmablastic proliferation limited to spleen. Eight cases of multicentric Castleman disease had a previously unrecognized presentation shortly after the diagnosis of HIV infection, six cases had cavity effusions, and three showed plasmablast enriched proliferations. One germinotropic lymphoproliferative disorder was EBV negative and three occurred in HIV-positive patients, who had distinctive clinical and morphological features. Two of the HHV8-related lymphomas did not fulfill the criteria for previously recognized entities. All these findings expand the clinical and pathological spectrum of HHV8-related lymphoid proliferations, which is broader than current recognized.
authors.affiliation: Department of Pathology, Hospital Clinic, Institut d'Investigacions Biomediques August Pi I Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain.
authors.forename: Blanca
authors.initials: B
authors.lastname: Gonzalez-Farre
authors.affiliation: Department of Pathology, Hospital Clinic, Institut d'Investigacions Biomediques August Pi I Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain.
authors.forename: Daniel
authors.initials: D
authors.lastname: Martinez
authors.affiliation: Department of Pathology, Hospital Clinic, Institut d'Investigacions Biomediques August Pi I Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain.
authors.forename: Monica
authors.initials: M
authors.lastname: Lopez-Guerra
authors.affiliation: Department of Geriatrics, Hospital Clinic, University of Barcelona, Barcelona, Spain.
authors.forename: Marc
authors.initials: M
authors.lastname: Xipell
authors.affiliation: Department of Geriatrics, Hospital Clinic, University of Barcelona, Barcelona, Spain.
authors.forename: Ester
authors.initials: E
authors.lastname: Monclus
authors.affiliation: Department of Hemathology, Hospital Clinic, Institut d'Investigacions Biomediques August Pi I Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain.
authors.forename: Jordina
authors.initials: J
authors.lastname: Rovira
authors.affiliation: Department of Infectious Diseases, Institut d'Investigacions Biomediques August Pi I Sunyer (IDIBAPS), Hospital Clinic, University of Barcelona, Barcelona, Spain.
authors.forename: Felipe
authors.initials: F
authors.lastname: Garcia
authors.affiliation: Department of Hemathology, Hospital Clinic, Institut d'Investigacions Biomediques August Pi I Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain.
authors.forename: Armando
authors.initials: A
authors.lastname: Lopez-Guillermo
authors.affiliation: Department of Pathology, Hematopathology Section, Hospital del Mar, Institut Mar d'Investigacions Mèdiques (IMIM), University Pompeu Fabra, Barcelona, Spain.
authors.forename: Luis
authors.initials: L
authors.lastname: Colomo
authors.affiliation: Department of Pathology, Hospital Clinic, Institut d'Investigacions Biomediques August Pi I Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain.
authors.forename: Elias
authors.identifier: 0000-0001-9850-9793
authors.initials: E
authors.lastname: Campo
authors.affiliation: Department of Pathology, Hospital Clinic, Institut d'Investigacions Biomediques August Pi I Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain.
authors.forename: Antonio
authors.initials: A
authors.lastname: Martinez
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D006566:Herpesviridae Infections / Q000150:complications* / Q000473:pathology; D019288:Herpesvirus 8, Human; D006801:Humans; D008223:Lymphoma / Q000473:pathology / Q000821:virology*; D008232:Lymphoproliferative Disorders / Q000473:pathology / Q000821:virology*; D008297:Male; D008875:Middle Aged; D055815:Young Adult