U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

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

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
Cardiac Lymphoma.
Jeudy J, Burke AP, Frazier AA
Radiol Clin North Am 2016 Jul;54(4):689-710. doi: 10.1016/j.rcl.2016.03.006. PMID: 27265603

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
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
HIV-associated lymphomas and gamma-herpesviruses.
Carbone A, Cesarman E, Spina M, Gloghini A, Schulz TF
Blood 2009 Feb 5;113(6):1213-24. Epub 2008 Oct 27 doi: 10.1182/blood-2008-09-180315. PMID: 18955561
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
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
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
Human herpesvirus-8: Kaposi sarcoma, multicentric Castleman disease, and primary effusion lymphoma.
Kaplan LD
Hematology Am Soc Hematol Educ Program 2013;2013:103-8. doi: 10.1182/asheducation-2013.1.103. PMID: 24319170

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

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Reviews

    Related information

    • MeSH
      Related Medical Subject Headings
    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...