Primary effusion lymphoma: a distinct clinicopathologic entity associated with the Kaposi's sarcoma-associated herpes virus.

Important note: Information in this article was accurate in 1996. The state of the art may have changed since the publication date.

Primary effusion lymphoma: a distinct clinicopathologic entity associated with the Kaposi's sarcoma-associated herpes virus.

Blood. 1996 Jul 15;88(2):645-56. Unique Identifier : AIDSLINE MED/96289513
Nador RG; Cesarman E; Chadburn A; Dawson DB; Ansari MQ; Sald J; Knowles DM; Department of Pathology, New York Hospital-Cornell Medical; Center, New York 10021, USA.

Abstract: We recently discovered the Kaposi's sarcoma-associated herpes virus (KSHV/HHV-8) in an uncommon and unusual subset of AIDS-related lymphomas that grow mainly in the body cavities as lymphomatous effusions without an identifiable contiguous tumor mass. The consistent presence of KSHV and certain other distinctive features of these body cavity-based lymphomas suggest that they represent a distinct entity. We tested this hypothesis by investigating 19 malignant lymphomatous effusions occurring in the absence of a contiguous tumor mass for their clinical, morphologic, immunophenotypic, viral, and molecular characteristics, KSHV was present in 15 of 19 lymphomas. All four KSHV-negative lymphomatous effusions exhibited Burkitt or Burkitt-like morphology and c-myc gene rearrangements and, therefore, appeared to be Burkitt-type lymphomas occurring in the body cavities. In contrast, all 15 KSHV-positive lymphomatous effusions exhibited a distinctive morphology bridging large-cell immunoblastic lymphoma and anaplastic large-cell lymphoma, and all 12 cases studied lacked c-myc gene rearrangements. In addition, these lymphomas occurred in men (15/15), frequently but not exclusively in association with HIV infection (13/15), in which homosexuality was a risk factor (13/13), presented initially as a lymphomatous effusion (14/15), remained localized to the body cavity of origin (13/15), expressed CD45 (15/15) and one or more activation-associated antigens (9/10) in the frequent absence of B-cell-associated antigens (11/15), exhibited clonal immunoglobulin gene rearrangements (13/13), contained Epstein-Barr virus (14/15), and lacked bcl-2, bcl-6, ras and p53 gene alterations (13/15). These findings strongly suggest that the KSHV-positive malignant lymphomatous effusions represent a distinct clinicopathologic and biologic entity and should be distinguished from other malignant lymphomas occurring in the body cavities. Therefore, we recommend that these malignant lymphomas be designated primary effusion lymphomas (PEL), rather than body cavity-based lymphomas, since this term describes them more accurately and avoids their confusion with other malignant lymphomas that occur in the body cavities. We further recommend that these PEL be considered for inclusion as a new entity in the Revised European-American Lymphoma Classification.

Keywords: Acquired Immunodeficiency Syndrome/*COMPLICATIONS Adult Aged Antigens, CD45/ANALYSIS Antigens, Differentiation, B-Lymphocyte/ANALYSIS Antigens, Neoplasm/ANALYSIS Ascites/*ETIOLOGY/PATHOLOGY/VIROLOGY Burkitt's Lymphoma/GENETICS/PATHOLOGY Comparative Study Gene Rearrangement Herpesviridae/*ISOLATION & PURIF/PATHOGENICITY Herpesviridae Infections/*COMPLICATIONS/VIROLOGY Herpesvirus 4, Human/ISOLATION & PURIF Homosexuality, Male Human Lymphoma, AIDS-Related/CLASSIFICATION/GENETICS/*PATHOLOGY/ VIROLOGY Lymphoma, Large-Cell/CLASSIFICATION/GENETICS/*PATHOLOGY/VIROLOGY Lymphoma, Large-Cell, Immunoblastic/CLASSIFICATION/GENETICS/ PATHOLOGY/VIROLOGY Male Middle Age Oncogenes Pleural Effusion, Malignant/*ETIOLOGY/PATHOLOGY/VIROLOGY Risk Factors Tumor Virus Infections/*COMPLICATIONS/VIROLOGY JOURNAL ARTICLE
961130
M96B1874

AEGiS is a 501(c)3, not-for-profit, tax-exempt, educational corporation. AEGiS is made possible through unrestricted funding from Boehringer Ingelheim, Bridgestone/Firestone Charitable Trust, Bristol-Myers Squibb Company, Elton John AIDS Foundation, Gill Foundation, the National Library of Medicine, Quest Diagnostics, Roche and Trimeris, and donations from users like you. Always watch for outdated information. This article first appeared in 1996. This material is designed to support, not replace, the relationship that exists between you and your doctor.

AEGiS presents published material, reprinted with permission and neither endorses nor opposes any material. All information contained on this website, including information relating to health conditions, products, and treatments, is for informational purposes only. It is often presented in summary or aggregate form. It is not meant to be a substitute for the advice provided by your own physician or other medical professionals. Always discuss treatment options with a doctor who specializes in treating HIV.

Copyright ©1980, 1996. AEGiS. All materials appearing on AEGiS are protected by copyright as a collective work or compilation under U.S. copyright and other laws and are the property of AEGiS, or the party credited as the provider of the content. .