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Primary effusion lymphoma(PEL)

MedGen UID:
220953
•Concept ID:
C1292753
•
Neoplastic Process
Synonyms: AIDS-related lymphoma; PEL
SNOMED CT: Primary effusion lymphoma (128800006); Primary effusion lymphoma (713516007)
 
Monarch Initiative: MONDO:0018842
Orphanet: ORPHA48686

Definition

Primary effusion lymphoma (PEL) is a large B-cell lymphoma located in the body cavities, characterized by pleural, peritoneal, and pericardial fluid lymphomatous effusions and that is always associated with human herpes virus-8 (HHV-8). [from ORDO]

Professional guidelines

PubMed

EBV-positive diffuse large B-cell lymphoma, not otherwise specified: 2022 update on diagnosis, risk-stratification, and management.
Malpica L, Marques-Piubelli ML, Beltran BE, Chavez JC, Miranda RN, Castillo JJ
Am J Hematol 2022 Jul;97(7):951-965. Epub 2022 May 3 doi: 10.1002/ajh.26579. PMID: 35472248
EBV-positive diffuse large B-cell lymphoma, not otherwise specified: 2018 update on diagnosis, risk-stratification and management.
Castillo JJ, Beltran BE, Miranda RN, Young KH, Chavez JC, Sotomayor EM
Am J Hematol 2018 Jul;93(7):953-962. doi: 10.1002/ajh.25112. PMID: 29984868
Primary effusion lymphoma: current concepts and management.
Arora N, Gupta A, Sadeghi N
Curr Opin Pulm Med 2017 Jul;23(4):365-370. doi: 10.1097/MCP.0000000000000384. PMID: 28399009

Recent clinical studies

Etiology

EBV-positive diffuse large B-cell lymphoma, not otherwise specified: 2022 update on diagnosis, risk-stratification, and management.
Malpica L, Marques-Piubelli ML, Beltran BE, Chavez JC, Miranda RN, Castillo JJ
Am J Hematol 2022 Jul;97(7):951-965. Epub 2022 May 3 doi: 10.1002/ajh.26579. PMID: 35472248
Castleman disease.
Carbone A, Borok M, Damania B, Gloghini A, Polizzotto MN, Jayanthan RK, Fajgenbaum DC, Bower M
Nat Rev Dis Primers 2021 Nov 25;7(1):84. doi: 10.1038/s41572-021-00317-7. PMID: 34824298Free PMC Article
AIDS-Associated Malignancies.
Vangipuram R, Tyring SK
Cancer Treat Res 2019;177:1-21. doi: 10.1007/978-3-030-03502-0_1. PMID: 30523619
EBV-positive diffuse large B-cell lymphoma, not otherwise specified: 2018 update on diagnosis, risk-stratification and management.
Castillo JJ, Beltran BE, Miranda RN, Young KH, Chavez JC, Sotomayor EM
Am J Hematol 2018 Jul;93(7):953-962. doi: 10.1002/ajh.25112. PMID: 29984868
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

EBV-positive diffuse large B-cell lymphoma, not otherwise specified: 2022 update on diagnosis, risk-stratification, and management.
Malpica L, Marques-Piubelli ML, Beltran BE, Chavez JC, Miranda RN, Castillo JJ
Am J Hematol 2022 Jul;97(7):951-965. Epub 2022 May 3 doi: 10.1002/ajh.26579. PMID: 35472248
Castleman disease.
Carbone A, Borok M, Damania B, Gloghini A, Polizzotto MN, Jayanthan RK, Fajgenbaum DC, Bower M
Nat Rev Dis Primers 2021 Nov 25;7(1):84. doi: 10.1038/s41572-021-00317-7. PMID: 34824298Free PMC Article
Diffuse large B-cell lymphoma variants: an update.
Sukswai N, Lyapichev K, Khoury JD, Medeiros LJ
Pathology 2020 Jan;52(1):53-67. Epub 2019 Nov 15 doi: 10.1016/j.pathol.2019.08.013. PMID: 31735345
AIDS-Associated Malignancies.
Vangipuram R, Tyring SK
Cancer Treat Res 2019;177:1-21. doi: 10.1007/978-3-030-03502-0_1. PMID: 30523619
Lymphoproliferative Disorders of the Lung.
Borie R, Wislez M, Antoine M, Cadranel J
Respiration 2017;94(2):157-175. Epub 2017 Jun 14 doi: 10.1159/000477740. PMID: 28609772

Therapy

Castleman disease.
Carbone A, Borok M, Damania B, Gloghini A, Polizzotto MN, Jayanthan RK, Fajgenbaum DC, Bower M
Nat Rev Dis Primers 2021 Nov 25;7(1):84. doi: 10.1038/s41572-021-00317-7. PMID: 34824298Free PMC Article
The Changing Landscape of Lymphoma Associated with HIV Infection.
Hübel K
Curr Oncol Rep 2020 Aug 15;22(11):111. doi: 10.1007/s11912-020-00973-0. PMID: 32803474Free PMC Article
Primary effusion lymphoma: current concepts and management.
Arora N, Gupta A, Sadeghi N
Curr Opin Pulm Med 2017 Jul;23(4):365-370. doi: 10.1097/MCP.0000000000000384. PMID: 28399009
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

Prognosis

EBV-positive diffuse large B-cell lymphoma, not otherwise specified: 2022 update on diagnosis, risk-stratification, and management.
Malpica L, Marques-Piubelli ML, Beltran BE, Chavez JC, Miranda RN, Castillo JJ
Am J Hematol 2022 Jul;97(7):951-965. Epub 2022 May 3 doi: 10.1002/ajh.26579. PMID: 35472248
Castleman disease.
Carbone A, Borok M, Damania B, Gloghini A, Polizzotto MN, Jayanthan RK, Fajgenbaum DC, Bower M
Nat Rev Dis Primers 2021 Nov 25;7(1):84. doi: 10.1038/s41572-021-00317-7. PMID: 34824298Free PMC Article
Primary effusion lymphoma enhancer connectome links super-enhancers to dependency factors.
Wang C, Zhang L, Ke L, Ding W, Jiang S, Li D, Narita Y, Hou I, Liang J, Li S, Xiao H, Gottwein E, Kaye KM, Teng M, Zhao B
Nat Commun 2020 Dec 9;11(1):6318. doi: 10.1038/s41467-020-20136-w. PMID: 33298918Free PMC Article
AIDS-Associated Malignancies.
Vangipuram R, Tyring SK
Cancer Treat Res 2019;177:1-21. doi: 10.1007/978-3-030-03502-0_1. PMID: 30523619
Lymphoproliferative Disorders of the Lung.
Borie R, Wislez M, Antoine M, Cadranel J
Respiration 2017;94(2):157-175. Epub 2017 Jun 14 doi: 10.1159/000477740. PMID: 28609772

Clinical prediction guides

EBV-positive diffuse large B-cell lymphoma, not otherwise specified: 2022 update on diagnosis, risk-stratification, and management.
Malpica L, Marques-Piubelli ML, Beltran BE, Chavez JC, Miranda RN, Castillo JJ
Am J Hematol 2022 Jul;97(7):951-965. Epub 2022 May 3 doi: 10.1002/ajh.26579. PMID: 35472248
Clinical Characteristics and Survival Outcomes of Primary Effusion Lymphoma: A National Cancer Database Study.
Baidoun F, Moustafa MA, Tun HW, Hill BT
Clin Lymphoma Myeloma Leuk 2022 Jul;22(7):e485-e494. Epub 2022 Jan 14 doi: 10.1016/j.clml.2022.01.006. PMID: 35110006
HIV-Related Lymphoproliferative Diseases in the Era of Combination Antiretroviral Therapy.
Castelli R, Schiavon R, Preti C, Ferraris L
Cardiovasc Hematol Disord Drug Targets 2020;20(3):175-180. doi: 10.2174/1871529X20666200415121009. PMID: 32294049Free PMC Article
EBV-positive diffuse large B-cell lymphoma, not otherwise specified: 2018 update on diagnosis, risk-stratification and management.
Castillo JJ, Beltran BE, Miranda RN, Young KH, Chavez JC, Sotomayor EM
Am J Hematol 2018 Jul;93(7):953-962. doi: 10.1002/ajh.25112. PMID: 29984868
Pathological Features of Kaposi's Sarcoma-Associated Herpesvirus Infection.
Katano H
Adv Exp Med Biol 2018;1045:357-376. doi: 10.1007/978-981-10-7230-7_16. PMID: 29896675

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
Germinotropic lymphoproliferative disorder: a systematic review.
Zanelli M, Zizzo M, Bisagni A, Froio E, De Marco L, Valli R, Filosa A, Luminari S, Martino G, Massaro F, Fratoni S, Ascani S
Ann Hematol 2020 Oct;99(10):2243-2253. Epub 2020 Apr 19 doi: 10.1007/s00277-020-04024-3. PMID: 32307569
CD20-negative diffuse large B cell lymphoma: a comprehensive analysis of 695 cases.
Li J, Zhao S, Wang J, Chen J, Wen W, Zhang Q
Tumour Biol 2016 Mar;37(3):3619-37. Epub 2015 Oct 12 doi: 10.1007/s13277-015-4205-5. PMID: 26459310
HHV-8 seroprevalence: a global view.
Rohner E, Wyss N, Trelle S, Mbulaiteye SM, Egger M, Novak U, Zwahlen M, Bohlius J
Syst Rev 2014 Feb 12;3:11. doi: 10.1186/2046-4053-3-11. PMID: 24521144Free PMC Article

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