{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,1]],"date-time":"2026-08-01T03:23:14Z","timestamp":1785554594391,"version":"3.56.0"},"reference-count":52,"publisher":"Archives of Pathology and Laboratory Medicine","issue":"8","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2013,8]]},"abstract":"<jats:p>Primary effusion lymphoma is a large cell non-Hodgkin lymphoma localized predominantly in body cavities and occasionally in extracavitary regions. It presents with characteristic lymphomatous effusions in the absence of solid tumor masses, and pleural, peritoneal, and pericardial spaces are most often involved. It is typically associated with human herpesvirus 8 infection in immunocompromised individuals, in the setting of human immunodeficiency virus infection, organ transplantation, or in rare cases advanced age. Histologically, primary effusion lymphoma is characterized by atypical lymphoid cells of B-cell lineage with large nuclei and prominent nucleoli. Demonstration of human herpesvirus 8 latent antigens is required for diagnosis, and treatment modalities are limited at this time. In this review, we aim to summarize clinicopathologic features of this rare and unique entity.<\/jats:p>","DOI":"10.5858\/arpa.2012-0294-rs","type":"journal-article","created":{"date-parts":[[2013,7,30]],"date-time":"2013-07-30T16:10:06Z","timestamp":1375200606000},"page":"1152-1154","source":"Crossref","is-referenced-by-count":40,"title":["Primary Effusion Lymphoma"],"prefix":"10.5858","volume":"137","author":[{"given":"Sanjay","family":"Patel","sequence":"first","affiliation":[{"name":"From the Department of Pathology and Laboratory Medicine, The Brooklyn Hospital Center, Brooklyn, New York."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philip","family":"Xiao","sequence":"additional","affiliation":[{"name":"From the Department of Pathology and Laboratory Medicine, The Brooklyn Hospital Center, Brooklyn, New York."}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"3906","reference":[{"key":"i1543-2165-137-8-1152-b01","series-title":"Pathology and Genetics of Haematopoietic and Lymphoid Tissues","first-page":"179","article-title":"Primary effusion lymphoma","author":"Banks\n              PW,","year":"2001","unstructured":"Banks\n              PW, \n            \n              Warnke\n              RA. \n          \n          Primary effusion lymphoma. In: Pathology and Genetics of Haematopoietic and Lymphoid Tissues. Lyon, France: IARC Press;2001:179\u2013180. WHO Classification of Tumours; vol 3."},{"key":"i1543-2165-137-8-1152-b02","series-title":"WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues","first-page":"260","article-title":"Primary effusion lymphoma","author":"Said\n              J,","year":"2008","unstructured":"Said\n              J, \n            \n              Cesarman\n              E. \n          \n          Primary effusion lymphoma. In: WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. Lyon, France: IARC Press;2008:260\u2013261. WHO Classification of Tumours; vol 2."},{"issue":"4","key":"i1543-2165-137-8-1152-b03","series-title":"Cancer Cytopathol","first-page":"225","article-title":"and HHV8-unrelated effusion lymphomas: classification and diagnosis","volume":"114","author":"Carbone\n              A,","year":"2008","unstructured":"Carbone\n              A, \n            \n              Gloghini\n              A. PEL\n            \n          \n          and HHV8-unrelated effusion lymphomas: classification and diagnosis. Cancer Cytopathol. 2008;114(4):225\u2013227."},{"issue":"4","key":"i1543-2165-137-8-1152-b04","series-title":"Cancer Cytopathol","first-page":"224","article-title":"Primary effusion lymphoma: a series of 4 cases and review of the literature with emphasis on cytomorphologic and immunocytochemical differential diagnosis","volume":"111","author":"Brimo\n              F,","year":"2007","unstructured":"Brimo\n              F, \n            \n              Michael\n              R, \n            \n              Khetani\n              K, \n            \n              Auger\n              M. \n          \n          Primary effusion lymphoma: a series of 4 cases and review of the literature with emphasis on cytomorphologic and immunocytochemical differential diagnosis. Cancer Cytopathol. 2007;111(4):224\u2013233."},{"issue":"9","key":"i1543-2165-137-8-1152-b05","series-title":"Arch Pathol Lab Med","first-page":"1246","article-title":"Association of primary pleural effusion lymphoma of T-cell origin and human herpesvirus 8 in a human immunodeficiency virus\u2013seronegative man","volume":"125","author":"Lechapt-Zalcman\n              E,","year":"2001","unstructured":"Lechapt-Zalcman\n              E, \n            \n              Challine\n              D, \n            \n              Delfau-Larue\n              MH, \n            \n              Haioun\n              C, \n            \n              Desvaux\n              D, \n            \n              Gaulard\n              P. \n          \n          Association of primary pleural effusion lymphoma of T-cell origin and human herpesvirus 8 in a human immunodeficiency virus\u2013seronegative man. Arch Pathol Lab Med. 2001;125(9):1246\u20131248."},{"issue":"3","key":"i1543-2165-137-8-1152-b06","series-title":"Acta Cytol","first-page":"425","article-title":"Human herpesvirus 8 in primary effusion lymphoma in an HIV-seronegative male: a case report","volume":"48","author":"Munichor\n              M,","year":"2004","unstructured":"Munichor\n              M, \n            \n              Cohen\n              H, \n            \n              Sarid\n              R, \n            \n              Manov\n              I, \n            \n              Iancu\n              TC. \n          \n          Human herpesvirus 8 in primary effusion lymphoma in an HIV-seronegative male: a case report. Acta Cytol. 2004;48(3):425\u2013430."},{"issue":"5","key":"i1543-2165-137-8-1152-b07","series-title":"Arch Pathol Lab Med","first-page":"753","article-title":"Early peripheral lymph node involvement of human herpesvirus 8\u2013associated, body cavity\u2013based lymphoma in a human immunodeficiency virus\u2013negative patient","volume":"124","author":"Ariad\n              S,","year":"2000","unstructured":"Ariad\n              S, \n            \n              Benharroch\n              D, \n            \n              Lupu\n              L, \n            \n              Davidovici\n              B, \n            \n              Dupin\n              N, \n            \n              Boshoff\n              C. \n          \n          Early peripheral lymph node involvement of human herpesvirus 8\u2013associated, body cavity\u2013based lymphoma in a human immunodeficiency virus\u2013negative patient. Arch Pathol Lab Med. 2000;124(5):753\u2013755."},{"issue":"5","key":"i1543-2165-137-8-1152-b08","series-title":"Eur Respir J","first-page":"1231","article-title":"Human herpes virus-8 associated primary effusion lymphoma of the pleural cavity in HIV-negative elderly men","volume":"14","author":"Ascoli\n              V,","year":"1999","unstructured":"Ascoli\n              V, \n            \n              Scalzo\n              CC, \n            \n              Danese\n              C, \n            \n              Vacca\n              K, \n            \n              Pistilli\n              A, \n            \n              Lo Coco\n              F. \n          \n          Human herpes virus-8 associated primary effusion lymphoma of the pleural cavity in HIV-negative elderly men. Eur Respir J. 1999;14(5):1231\u20131234."},{"issue":"1","key":"i1543-2165-137-8-1152-b09","series-title":"J Mol Diagn","first-page":"17","article-title":"Kaposi's sarcoma-associated herpesvirus\/human herpesvirus type 8-positive solid lymphomas: a tissue-based variant of primary effusion lymphoma","volume":"7","author":"Carbone\n              A,","year":"2005","unstructured":"Carbone\n              A, \n            \n              Gloghini\n              A, \n            \n              Vaccher\n              E, \n            et al\n          . Kaposi's sarcoma-associated herpesvirus\/human herpesvirus type 8-positive solid lymphomas: a tissue-based variant of primary effusion lymphoma. J Mol Diagn. 2005;7(1):17\u201327."},{"issue":"11","key":"i1543-2165-137-8-1152-b10","series-title":"Am J Surg Pathol","first-page":"1401","article-title":"KSHV-positive solid lymphomas represent an extra-cavitary variant of primary effusion lymphoma","volume":"28","author":"Chadburn\n              A,","year":"2004","unstructured":"Chadburn\n              A, \n            \n              Hyjek\n              E, \n            \n              Mathew\n              S, \n            \n              Cesarman\n              E, \n            \n              Said\n              J, \n            \n              Knowles\n              DM. \n          \n          KSHV-positive solid lymphomas represent an extra-cavitary variant of primary effusion lymphoma. Am J Surg Pathol. 2004;28(11):1401\u20131416."},{"issue":"3","key":"i1543-2165-137-8-1152-b11","series-title":"Blood","first-page":"744","article-title":"Expression profile of MUM1\/IRF4, BCL-6, and CD138\/syndecan-1 defines novel histogenetic subsets of human immunodeficiency virus-related lymphomas","volume":"97","author":"Carbone\n              A,","year":"2001","unstructured":"Carbone\n              A, \n            \n              Gloghini\n              A, \n            \n              Larocca\n              LM, \n            et al\n          . Expression profile of MUM1\/IRF4, BCL-6, and CD138\/syndecan-1 defines novel histogenetic subsets of human immunodeficiency virus-related lymphomas. Blood. 2001;97(3):744\u2013751."},{"issue":"10","key":"i1543-2165-137-8-1152-b12","series-title":"Blood","first-page":"4115","article-title":"Gene expression profile analysis of AIDS-related primary effusion lymphoma (PEL) suggests a plasmablastic derivation and identifies PEL-specific transcripts","volume":"101","author":"Klein\n              U,","year":"2003","unstructured":"Klein\n              U, \n            \n              Gloghini\n              A, \n            \n              Gaidano\n              G, \n            et al\n          . Gene expression profile analysis of AIDS-related primary effusion lymphoma (PEL) suggests a plasmablastic derivation and identifies PEL-specific transcripts. Blood. 2003;101(10):4115\u20134121."},{"issue":"2","key":"i1543-2165-137-8-1152-b13","series-title":"Blood","first-page":"645","article-title":"Primary effusion lymphoma: a distinct clinicopathologic entity associated with the Kaposi's sarcoma-associated herpes virus","volume":"88","author":"Nador\n              RG,","year":"1996","unstructured":"Nador\n              RG, \n            \n              Cesarman\n              E, \n            \n              Chadburn\n              A, \n            et al\n          . Primary effusion lymphoma: a distinct clinicopathologic entity associated with the Kaposi's sarcoma-associated herpes virus. Blood. 1996;88(2):645\u2013656."},{"issue":"18","key":"i1543-2165-137-8-1152-b14","series-title":"N Engl J Med","first-page":"1186","article-title":"Kaposi's sarcoma-associated herpesvirus-like DNA sequences in AIDS-related body-cavity-based lymphomas","volume":"332","author":"Cesarman\n              E,","year":"1995","unstructured":"Cesarman\n              E, \n            \n              Chang\n              Y, \n            \n              Moore\n              PS, \n            \n              Said\n              JW, \n            \n              Knowles\n              DM. \n          \n          Kaposi's sarcoma-associated herpesvirus-like DNA sequences in AIDS-related body-cavity-based lymphomas. N Engl J Med. 1995;332(18):1186\u20131191."},{"issue":"4","key":"i1543-2165-137-8-1152-b15","series-title":"Am J Surg Pathol","first-page":"493","article-title":"Molecular and pathologic characterization of an AIDS-related body cavity-based lymphoma, including ultrastructural demonstration of human herpesvirus-8: a case report","volume":"22","author":"Hsi\n              ED,","year":"1998","unstructured":"Hsi\n              ED, \n            \n              Foreman\n              KE, \n            \n              Duggan\n              J, \n            et al\n          . Molecular and pathologic characterization of an AIDS-related body cavity-based lymphoma, including ultrastructural demonstration of human herpesvirus-8: a case report. Am J Surg Pathol. 1998;22(4):493\u2013499."},{"issue":"9","key":"i1543-2165-137-8-1152-b16","series-title":"Mod Pathol","first-page":"944","article-title":"Primary effusion lymphoma: cytopathologic diagnosis using in situ molecular genetic analysis for human herpesvirus 8","volume":"15","author":"Wakely\n              PE\n              Jr,","year":"2002","unstructured":"Wakely\n              PE\n              Jr, \n            \n              Menezes\n              G, \n            \n              Nuovo\n              GJ. \n          \n          Primary effusion lymphoma: cytopathologic diagnosis using in situ molecular genetic analysis for human herpesvirus 8. Mod Pathol. 2002;15(9):944\u2013950."},{"issue":"20","key":"i1543-2165-137-8-1152-b17","series-title":"J Clin Oncol","first-page":"4255","article-title":"Pyothorax-associated lymphoma: a review of 106 cases","volume":"20","author":"Nakatsuka\n              S-I,","year":"2002","unstructured":"Nakatsuka\n              S-I, \n            \n              Yao\n              M, \n            \n              Hoshida\n              Y, \n            \n              Yamamoto\n              S, \n            \n              Iuchi\n              K, \n            \n              Aozasa\n              K. \n          \n          Pyothorax-associated lymphoma: a review of 106 cases. J Clin Oncol. 2002;20(20):4255\u20134260."},{"issue":"4","key":"i1543-2165-137-8-1152-b18","series-title":"Blood","first-page":"1413","article-title":"Plasmablastic lymphomas of the oral cavity: a new entity associated with the human immunodeficiency virus infection","volume":"89","author":"Delecluse\n              HJ,","year":"1997","unstructured":"Delecluse\n              HJ, \n            \n              Anagnostopoulos\n              I, \n            \n              Dallenbach\n              F, \n            et al\n          . Plasmablastic lymphomas of the oral cavity: a new entity associated with the human immunodeficiency virus infection. Blood. 1997;89(4):1413\u20131420."},{"issue":"11","key":"i1543-2165-137-8-1152-b19","series-title":"Blood","first-page":"3909","volume":"89","year":"1997","unstructured":"A clinical evaluation of the International Lymphoma Study Group classification of non-Hodgkin's lymphoma: the Non-Hodgkin's Lymphoma Classification Project. 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Blood. 2000;96(12):3681\u20133695."},{"issue":"5196","key":"i1543-2165-137-8-1152-b21","series-title":"Science","first-page":"316","article-title":"Fusion of a kinase gene, ALK, to a nucleolar protein gene, NPM, in non-Hodgkin's lymphoma","volume":"267","author":"Morris\n              SW,","year":"1995","unstructured":"Morris\n              SW, \n            \n              Kirstein\n              MN, \n            \n              Valentine\n              MB, \n            et al\n          . Fusion of a kinase gene, ALK, to a nucleolar protein gene, NPM, in non-Hodgkin's lymphoma. 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In: WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. Lyon, France: IARC Press;2008:260\u2013261. WHO Classification of Tumours; vol 2."},{"issue":"4","key":"i1543-2165-137-8-1152-b03","series-title":"Cancer Cytopathol","first-page":"225","article-title":"and HHV8-unrelated effusion lymphomas: classification and diagnosis","volume":"114","author":"Carbone\n              A,","year":"2008","unstructured":"Carbone\n              A, \n            \n              Gloghini\n              A. PEL\n            \n          \n          and HHV8-unrelated effusion lymphomas: classification and diagnosis. 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Cancer Cytopathol. 2007;111(4):224\u2013233."},{"issue":"9","key":"i1543-2165-137-8-1152-b05","series-title":"Arch Pathol Lab Med","first-page":"1246","article-title":"Association of primary pleural effusion lymphoma of T-cell origin and human herpesvirus 8 in a human immunodeficiency virus\u2013seronegative man","volume":"125","author":"Lechapt-Zalcman\n              E,","year":"2001","unstructured":"Lechapt-Zalcman\n              E, \n            \n              Challine\n              D, \n            \n              Delfau-Larue\n              MH, \n            \n              Haioun\n              C, \n            \n              Desvaux\n              D, \n            \n              Gaulard\n              P. \n          \n          Association of primary pleural effusion lymphoma of T-cell origin and human herpesvirus 8 in a human immunodeficiency virus\u2013seronegative man. 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Arch Pathol Lab Med. 2000;124(5):753\u2013755."},{"issue":"5","key":"i1543-2165-137-8-1152-b08","series-title":"Eur Respir J","first-page":"1231","article-title":"Human herpes virus-8 associated primary effusion lymphoma of the pleural cavity in HIV-negative elderly men","volume":"14","author":"Ascoli\n              V,","year":"1999","unstructured":"Ascoli\n              V, \n            \n              Scalzo\n              CC, \n            \n              Danese\n              C, \n            \n              Vacca\n              K, \n            \n              Pistilli\n              A, \n            \n              Lo Coco\n              F. \n          \n          Human herpes virus-8 associated primary effusion lymphoma of the pleural cavity in HIV-negative elderly men. Eur Respir J. 1999;14(5):1231\u20131234."},{"issue":"1","key":"i1543-2165-137-8-1152-b09","series-title":"J Mol Diagn","first-page":"17","article-title":"Kaposi's sarcoma-associated herpesvirus\/human herpesvirus type 8-positive solid lymphomas: a tissue-based variant of primary effusion lymphoma","volume":"7","author":"Carbone\n              A,","year":"2005","unstructured":"Carbone\n              A, \n            \n              Gloghini\n              A, \n            \n              Vaccher\n              E, \n            et al\n          . Kaposi's sarcoma-associated herpesvirus\/human herpesvirus type 8-positive solid lymphomas: a tissue-based variant of primary effusion lymphoma. J Mol Diagn. 2005;7(1):17\u201327."},{"issue":"11","key":"i1543-2165-137-8-1152-b10","series-title":"Am J Surg Pathol","first-page":"1401","article-title":"KSHV-positive solid lymphomas represent an extra-cavitary variant of primary effusion lymphoma","volume":"28","author":"Chadburn\n              A,","year":"2004","unstructured":"Chadburn\n              A, \n            \n              Hyjek\n              E, \n            \n              Mathew\n              S, \n            \n              Cesarman\n              E, \n            \n              Said\n              J, \n            \n              Knowles\n              DM. \n          \n          KSHV-positive solid lymphomas represent an extra-cavitary variant of primary effusion lymphoma. Am J Surg Pathol. 2004;28(11):1401\u20131416."},{"issue":"3","key":"i1543-2165-137-8-1152-b11","series-title":"Blood","first-page":"744","article-title":"Expression profile of MUM1\/IRF4, BCL-6, and CD138\/syndecan-1 defines novel histogenetic subsets of human immunodeficiency virus-related lymphomas","volume":"97","author":"Carbone\n              A,","year":"2001","unstructured":"Carbone\n              A, \n            \n              Gloghini\n              A, \n            \n              Larocca\n              LM, \n            et al\n          . Expression profile of MUM1\/IRF4, BCL-6, and CD138\/syndecan-1 defines novel histogenetic subsets of human immunodeficiency virus-related lymphomas. Blood. 2001;97(3):744\u2013751."},{"issue":"10","key":"i1543-2165-137-8-1152-b12","series-title":"Blood","first-page":"4115","article-title":"Gene expression profile analysis of AIDS-related primary effusion lymphoma (PEL) suggests a plasmablastic derivation and identifies PEL-specific transcripts","volume":"101","author":"Klein\n              U,","year":"2003","unstructured":"Klein\n              U, \n            \n              Gloghini\n              A, \n            \n              Gaidano\n              G, \n            et al\n          . Gene expression profile analysis of AIDS-related primary effusion lymphoma (PEL) suggests a plasmablastic derivation and identifies PEL-specific transcripts. Blood. 2003;101(10):4115\u20134121."},{"issue":"2","key":"i1543-2165-137-8-1152-b13","series-title":"Blood","first-page":"645","article-title":"Primary effusion lymphoma: a distinct clinicopathologic entity associated with the Kaposi's sarcoma-associated herpes virus","volume":"88","author":"Nador\n              RG,","year":"1996","unstructured":"Nador\n              RG, \n            \n              Cesarman\n              E, \n            \n              Chadburn\n              A, \n            et al\n          . Primary effusion lymphoma: a distinct clinicopathologic entity associated with the Kaposi's sarcoma-associated herpes virus. Blood. 1996;88(2):645\u2013656."},{"issue":"18","key":"i1543-2165-137-8-1152-b14","series-title":"N Engl J Med","first-page":"1186","article-title":"Kaposi's sarcoma-associated herpesvirus-like DNA sequences in AIDS-related body-cavity-based lymphomas","volume":"332","author":"Cesarman\n              E,","year":"1995","unstructured":"Cesarman\n              E, \n            \n              Chang\n              Y, \n            \n              Moore\n              PS, \n            \n              Said\n              JW, \n            \n              Knowles\n              DM. \n          \n          Kaposi's sarcoma-associated herpesvirus-like DNA sequences in AIDS-related body-cavity-based lymphomas. 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