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Body Cavity Primary Effusion Lymphoma

MedGen UID:
1669221
•Concept ID:
C4725708
•
Neoplastic Process
Synonym: Body Cavity PEL

Definition

A large B-cell lymphoma presenting as a serous effusion without detectable tumor masses. It is universally associated with human herpes virus 8 (HHV8), also called Kaposi sarcoma-associated herpesvirus. It mostly occurs in the setting of immunodeficiency. The most common sites of involvement are the pleural, pericardial, and peritoneal cavities. (WHO 2017) [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVBody Cavity Primary Effusion Lymphoma

Professional guidelines

PubMed

Biology and management of primary effusion lymphoma.
Shimada K, Hayakawa F, Kiyoi H
Blood 2018 Nov 1;132(18):1879-1888. Epub 2018 Aug 28 doi: 10.1182/blood-2018-03-791426. PMID: 30154110
Diagnosis and management of primary effusion lymphoma in the immunocompetent and immunocompromised hosts.
Ammari ZA, Mollberg NM, Abdelhady K, Mansueto MD, Massad MG
Thorac Cardiovasc Surg 2013 Jun;61(4):343-9. Epub 2013 Feb 19 doi: 10.1055/s-0033-1333897. PMID: 23424065
Primary effusion lymphoma: a series of 4 cases and review of the literature with emphasis on cytomorphologic and immunocytochemical differential diagnosis.
Brimo F, Michel RP, Khetani K, Auger M
Cancer 2007 Aug 25;111(4):224-33. doi: 10.1002/cncr.22691. PMID: 17554754

Recent clinical studies

Etiology

Human herpesvirus 8-negative effusion-based large B-cell lymphoma: a distinct entity with unique clinicopathologic characteristics.
Gisriel SD, Yuan J, Braunberger RC, Maracaja DLV, Chen X, Wu X, McCracken J, Chen M, Xie Y, Brown LE, Li P, Zhou Y, Sethi T, McHenry A, Hauser RG, Paulson N, Tang H, Hsi ED, Wang E, Zhang QY, Young KH, Xu ML, Pan Z
Mod Pathol 2022 Oct;35(10):1411-1422. Epub 2022 May 13 doi: 10.1038/s41379-022-01091-x. PMID: 35562413Free PMC Article
Primary human herpesvirus 8-negative effusion-based lymphoma: a large B-cell lymphoma with favorable prognosis.
Kaji D, Ota Y, Sato Y, Nagafuji K, Ueda Y, Okamoto M, Terasaki Y, Tsuyama N, Matsue K, Kinoshita T, Yamamoto G, Taniguchi S, Chiba S, Ohshima K, Izutsu K
Blood Adv 2020 Sep 22;4(18):4442-4450. doi: 10.1182/bloodadvances.2020002293. PMID: 32936906Free PMC Article
Clinicopathologic characteristics and survival of patients with primary effusion lymphoma.
Aguilar C, Laberiano C, Beltran B, Diaz C, Taype-Rondan A, Castillo JJ
Leuk Lymphoma 2020 Sep;61(9):2093-2102. Epub 2020 May 25 doi: 10.1080/10428194.2020.1762881. PMID: 32449626
Evidence for a Mesothelial Origin of Body Cavity Effusion Lymphomas.
Sanchez-Martin D, Uldrick TS, Kwak H, Ohnuki H, Polizzotto MN, Annunziata CM, Raffeld M, Wyvill KM, Aleman K, Wang V, Marshall VA, Whitby D, Yarchoan R, Tosato G
J Natl Cancer Inst 2017 Sep 1;109(9) doi: 10.1093/jnci/djx016. PMID: 28376153Free PMC Article
Body cavity lymphoma.
Ascoli V, Lo-Coco F
Curr Opin Pulm Med 2002 Jul;8(4):317-22. doi: 10.1097/00063198-200207000-00013. PMID: 12055396

Diagnosis

Primary effusion lymphoma.
Patel S, Xiao P
Arch Pathol Lab Med 2013 Aug;137(8):1152-4. doi: 10.5858/arpa.2012-0294-RS. PMID: 23899073
Diagnosis and management of primary effusion lymphoma in the immunocompetent and immunocompromised hosts.
Ammari ZA, Mollberg NM, Abdelhady K, Mansueto MD, Massad MG
Thorac Cardiovasc Surg 2013 Jun;61(4):343-9. Epub 2013 Feb 19 doi: 10.1055/s-0033-1333897. PMID: 23424065
Primary effusion lymphoma.
Chen YB, Rahemtullah A, Hochberg E
Oncologist 2007 May;12(5):569-76. doi: 10.1634/theoncologist.12-5-569. PMID: 17522245
Body cavity lymphoma.
Ascoli V, Lo-Coco F
Curr Opin Pulm Med 2002 Jul;8(4):317-22. doi: 10.1097/00063198-200207000-00013. PMID: 12055396
Immunodeficiency-associated lymphoproliferative disorders.
Knowles DM
Mod Pathol 1999 Feb;12(2):200-17. PMID: 10071343

Therapy

Spontaneous regression of dasatinib-related primary effusion lymphoma-like lymphoma.
Hayashino K, Meguri Y, Yukawa R, Komura A, Nakamura M, Yoshida C, Yamamoto K, Oda W, Imajo K
Int J Hematol 2023 Jan;117(1):137-142. Epub 2022 Sep 6 doi: 10.1007/s12185-022-03449-y. PMID: 36066839Free PMC Article
Efficacy and mechanism of the anti-CD38 monoclonal antibody Daratumumab against primary effusion lymphoma.
Panaampon J, Kariya R, Okada S
Cancer Immunol Immunother 2022 May;71(5):1017-1031. Epub 2021 Sep 20 doi: 10.1007/s00262-021-03054-8. PMID: 34545416Free PMC Article
Diagnosis and management of primary effusion lymphoma in the immunocompetent and immunocompromised hosts.
Ammari ZA, Mollberg NM, Abdelhady K, Mansueto MD, Massad MG
Thorac Cardiovasc Surg 2013 Jun;61(4):343-9. Epub 2013 Feb 19 doi: 10.1055/s-0033-1333897. PMID: 23424065
Primary effusion lymphoma.
Chen YB, Rahemtullah A, Hochberg E
Oncologist 2007 May;12(5):569-76. doi: 10.1634/theoncologist.12-5-569. PMID: 17522245
Immunodeficiency-associated lymphoproliferative disorders.
Knowles DM
Mod Pathol 1999 Feb;12(2):200-17. PMID: 10071343

Prognosis

Spontaneous regression of dasatinib-related primary effusion lymphoma-like lymphoma.
Hayashino K, Meguri Y, Yukawa R, Komura A, Nakamura M, Yoshida C, Yamamoto K, Oda W, Imajo K
Int J Hematol 2023 Jan;117(1):137-142. Epub 2022 Sep 6 doi: 10.1007/s12185-022-03449-y. PMID: 36066839Free PMC Article
Human herpesvirus 8-negative effusion-based large B-cell lymphoma: a distinct entity with unique clinicopathologic characteristics.
Gisriel SD, Yuan J, Braunberger RC, Maracaja DLV, Chen X, Wu X, McCracken J, Chen M, Xie Y, Brown LE, Li P, Zhou Y, Sethi T, McHenry A, Hauser RG, Paulson N, Tang H, Hsi ED, Wang E, Zhang QY, Young KH, Xu ML, Pan Z
Mod Pathol 2022 Oct;35(10):1411-1422. Epub 2022 May 13 doi: 10.1038/s41379-022-01091-x. PMID: 35562413Free PMC Article
Clinicopathologic characteristics and survival of patients with primary effusion lymphoma.
Aguilar C, Laberiano C, Beltran B, Diaz C, Taype-Rondan A, Castillo JJ
Leuk Lymphoma 2020 Sep;61(9):2093-2102. Epub 2020 May 25 doi: 10.1080/10428194.2020.1762881. PMID: 32449626
KSHV/HHV8-associated lymphomas.
Carbone A, Gloghini A
Br J Haematol 2008 Jan;140(1):13-24. Epub 2007 Nov 7 doi: 10.1111/j.1365-2141.2007.06879.x. PMID: 17991301
Primary effusion lymphoma.
Chen YB, Rahemtullah A, Hochberg E
Oncologist 2007 May;12(5):569-76. doi: 10.1634/theoncologist.12-5-569. PMID: 17522245

Clinical prediction guides

Primary human herpesvirus 8-negative effusion-based lymphoma: a large B-cell lymphoma with favorable prognosis.
Kaji D, Ota Y, Sato Y, Nagafuji K, Ueda Y, Okamoto M, Terasaki Y, Tsuyama N, Matsue K, Kinoshita T, Yamamoto G, Taniguchi S, Chiba S, Ohshima K, Izutsu K
Blood Adv 2020 Sep 22;4(18):4442-4450. doi: 10.1182/bloodadvances.2020002293. PMID: 32936906Free PMC Article
Primary Effusion Lymphoma-like Lymphoma in a Patient with Neurofibromatosis Type 1.
Oki M, Nanao T, Shinoda T, Tsuda A, Yasuda A, Seki T, Ozawa H, Nakamura N, Takagi A
Tokai J Exp Clin Med 2016 Sep 20;41(3):123-9. PMID: 27628603
Classic and extracavitary primary effusion lymphoma in 51 HIV-infected patients from a single institution.
Guillet S, Gérard L, Meignin V, Agbalika F, Cuccini W, Denis B, Katlama C, Galicier L, Oksenhendler E
Am J Hematol 2016 Feb;91(2):233-7. doi: 10.1002/ajh.24251. PMID: 26799611
Primary effusion lymphoma: a series of 4 cases and review of the literature with emphasis on cytomorphologic and immunocytochemical differential diagnosis.
Brimo F, Michel RP, Khetani K, Auger M
Cancer 2007 Aug 25;111(4):224-33. doi: 10.1002/cncr.22691. PMID: 17554754
Immunodeficiency and its relation to lymphoid and other malignancies.
Tossing G
Ann Hematol 1996 Oct;73(4):163-7. doi: 10.1007/s002770050221. PMID: 8890703

Recent systematic reviews

Primary effusion lymphoma occurring in the setting of transplanted patients: a systematic review of a rare, life-threatening post-transplantation occurrence.
Zanelli M, Sanguedolce F, Zizzo M, Palicelli A, Bassi MC, Santandrea G, Martino G, Soriano A, Caprera C, Corsi M, Ricci S, Ricci L, Ascani S
BMC Cancer 2021 Apr 27;21(1):468. doi: 10.1186/s12885-021-08215-7. PMID: 33906629Free PMC Article
Clinicopathologic characteristics and survival of patients with primary effusion lymphoma.
Aguilar C, Laberiano C, Beltran B, Diaz C, Taype-Rondan A, Castillo JJ
Leuk Lymphoma 2020 Sep;61(9):2093-2102. Epub 2020 May 25 doi: 10.1080/10428194.2020.1762881. PMID: 32449626

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