Primary effusion lymphoma

A distinct clinicopathologic entity associated with the Kaposi's sarcoma-associated herpes virus

Roland G. Nador, Ethel Cesarman, Amy Chadburn, D. Brian Dawson, M. Qasim Ansari, Jonathan Said, Daniel M. Knowles

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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 Burkite-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 contusion 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.

Original languageEnglish (US)
Pages (from-to)645-656
Number of pages12
JournalBlood
Volume88
Issue number2
StatePublished - Jul 15 1996

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Primary Effusion Lymphoma
Satellite Viruses
Kaposi's Sarcoma
Human Herpesvirus 8
Viruses
Lymphoma
Genes
Tumors
Gene Rearrangement
Antigens
myc Genes
Immunoglobulins
Chemical activation
Cells
Association reactions
Large-Cell Immunoblastic Lymphoma
AIDS-Related Lymphoma
Anaplastic Large-Cell Lymphoma
Immunoglobulin Genes
ras Genes

ASJC Scopus subject areas

  • Hematology

Cite this

Nador, R. G., Cesarman, E., Chadburn, A., Dawson, D. B., Ansari, M. Q., Said, J., & Knowles, D. M. (1996). Primary effusion lymphoma: A distinct clinicopathologic entity associated with the Kaposi's sarcoma-associated herpes virus. Blood, 88(2), 645-656.

Primary effusion lymphoma : A distinct clinicopathologic entity associated with the Kaposi's sarcoma-associated herpes virus. / Nador, Roland G.; Cesarman, Ethel; Chadburn, Amy; Dawson, D. Brian; Ansari, M. Qasim; Said, Jonathan; Knowles, Daniel M.

In: Blood, Vol. 88, No. 2, 15.07.1996, p. 645-656.

Research output: Contribution to journalArticle

Nador, RG, Cesarman, E, Chadburn, A, Dawson, DB, Ansari, MQ, Said, J & Knowles, DM 1996, 'Primary effusion lymphoma: A distinct clinicopathologic entity associated with the Kaposi's sarcoma-associated herpes virus', Blood, vol. 88, no. 2, pp. 645-656.
Nador RG, Cesarman E, Chadburn A, Dawson DB, Ansari MQ, Said J et al. Primary effusion lymphoma: A distinct clinicopathologic entity associated with the Kaposi's sarcoma-associated herpes virus. Blood. 1996 Jul 15;88(2):645-656.
Nador, Roland G. ; Cesarman, Ethel ; Chadburn, Amy ; Dawson, D. Brian ; Ansari, M. Qasim ; Said, Jonathan ; Knowles, Daniel M. / Primary effusion lymphoma : A distinct clinicopathologic entity associated with the Kaposi's sarcoma-associated herpes virus. In: Blood. 1996 ; Vol. 88, No. 2. pp. 645-656.
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