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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

Plasmablastic lymphoma: 2024 update on diagnosis, risk stratification, and management.
Ramirez-Gamero A, Martínez-Cordero H, Beltrán BE, Florindez J, Malpica L, Castillo JJ
Am J Hematol 2024 Aug;99(8):1586-1594. Epub 2024 May 20 doi: 10.1002/ajh.27376. PMID: 38767403
Clinical management of Kaposi sarcoma herpesvirus-associated diseases: an update on disease manifestations and treatment strategies.
Patel R, Lurain K, Yarchoan R, Ramaswami R
Expert Rev Anti Infect Ther 2023 Jul-Dec;21(9):929-941. Epub 2023 Aug 15 doi: 10.1080/14787210.2023.2247161. PMID: 37578202Free PMC Article
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

Recent clinical studies

Etiology

Plasmablastic lymphoma: 2024 update on diagnosis, risk stratification, and management.
Ramirez-Gamero A, Martínez-Cordero H, Beltrán BE, Florindez J, Malpica L, Castillo JJ
Am J Hematol 2024 Aug;99(8):1586-1594. Epub 2024 May 20 doi: 10.1002/ajh.27376. PMID: 38767403
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
Rhadinoviral interferon regulatory factor homologues.
Koch S, Schulz TF
Biol Chem 2017 Jul 26;398(8):857-870. doi: 10.1515/hsz-2017-0111. PMID: 28455950

Diagnosis

Plasmablastic lymphoma: 2024 update on diagnosis, risk stratification, and management.
Ramirez-Gamero A, Martínez-Cordero H, Beltrán BE, Florindez J, Malpica L, Castillo JJ
Am J Hematol 2024 Aug;99(8):1586-1594. Epub 2024 May 20 doi: 10.1002/ajh.27376. PMID: 38767403
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
Update on Kaposi sarcoma-associated herpesvirus (KSHV or HHV8) - review.
Iftode N, Rădulescu MA, Aramă ȘS, Aramă V
Rom J Intern Med 2020 Dec 1;58(4):199-208. Epub 2020 Dec 17 doi: 10.2478/rjim-2020-0017. PMID: 32681788
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

Therapy

Plasmablastic lymphoma: 2024 update on diagnosis, risk stratification, and management.
Ramirez-Gamero A, Martínez-Cordero H, Beltrán BE, Florindez J, Malpica L, Castillo JJ
Am J Hematol 2024 Aug;99(8):1586-1594. Epub 2024 May 20 doi: 10.1002/ajh.27376. PMID: 38767403
Druggable host gene dependencies in primary effusion lymphoma.
Kuehnle N, Gottwein E
Curr Opin Virol 2022 Oct;56:101270. Epub 2022 Sep 28 doi: 10.1016/j.coviro.2022.101270. PMID: 36182745Free PMC Article
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: 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
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

Plasmablastic lymphoma: 2024 update on diagnosis, risk stratification, and management.
Ramirez-Gamero A, Martínez-Cordero H, Beltrán BE, Florindez J, Malpica L, Castillo JJ
Am J Hematol 2024 Aug;99(8):1586-1594. Epub 2024 May 20 doi: 10.1002/ajh.27376. PMID: 38767403
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
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

Plasmablastic lymphoma: 2024 update on diagnosis, risk stratification, and management.
Ramirez-Gamero A, Martínez-Cordero H, Beltrán BE, Florindez J, Malpica L, Castillo JJ
Am J Hematol 2024 Aug;99(8):1586-1594. Epub 2024 May 20 doi: 10.1002/ajh.27376. PMID: 38767403
Co-Infection of the Epstein-Barr Virus and the Kaposi Sarcoma-Associated Herpesvirus.
Böni M, Rieble L, Münz C
Viruses 2022 Dec 2;14(12) doi: 10.3390/v14122709. PMID: 36560713Free PMC Article
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
Kaposi sarcoma-associated herpesvirus/human herpesvirus 8-associated lymphoproliferative disorders.
Oksenhendler E, Boutboul D, Galicier L
Blood 2019 Mar 14;133(11):1186-1190. Epub 2019 Jan 4 doi: 10.1182/blood-2018-11-852442. PMID: 30610029
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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