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Refractory Primary Effusion Lymphoma

MedGen UID:
1676531
•Concept ID:
C4763784
•
Neoplastic Process
Synonym: Refractory HHV8-Positive Primary Effusion Lymphoma

Definition

Primary effusion lymphoma that is resistant to treatment. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVRefractory Primary Effusion Lymphoma

Professional guidelines

PubMed

Treatment outcome and prognostic factors for primary mediastinal (thymic) B-cell lymphoma: a multicenter study of 106 patients.
Lazzarino M, Orlandi E, Paulli M, Sträter J, Klersy C, Gianelli U, Gargantini L, Rousset MT, Gambacorta M, Marra E, Lavabre-Bertrand T, Magrini U, Manegold C, Bernasconi C, Möller P
J Clin Oncol 1997 Apr;15(4):1646-53. doi: 10.1200/JCO.1997.15.4.1646. PMID: 9193365

Recent clinical studies

Etiology

Hematologic cancers in individuals infected by HIV.
Carbone A, Vaccher E, Gloghini A
Blood 2022 Feb 17;139(7):995-1012. doi: 10.1182/blood.2020005469. PMID: 34469512
Use of pembrolizumab with or without pomalidomide in HIV-associated non-Hodgkin's lymphoma.
Lurain K, Ramaswami R, Mangusan R, Widell A, Ekwede I, George J, Ambinder R, Cheever M, Gulley JL, Goncalves PH, Wang HW, Uldrick TS, Yarchoan R
J Immunother Cancer 2021 Feb;9(2) doi: 10.1136/jitc-2020-002097. PMID: 33608378Free PMC Article
Pembrolizumab in paediatric patients with advanced melanoma or a PD-L1-positive, advanced, relapsed, or refractory solid tumour or lymphoma (KEYNOTE-051): interim analysis of an open-label, single-arm, phase 1-2 trial.
Geoerger B, Kang HJ, Yalon-Oren M, Marshall LV, Vezina C, Pappo A, Laetsch TW, Petrilli AS, Ebinger M, Toporski J, Glade-Bender J, Nicholls W, Fox E, DuBois SG, Macy ME, Cohn SL, Pathiraja K, Diede SJ, Ebbinghaus S, Pinto N
Lancet Oncol 2020 Jan;21(1):121-133. Epub 2019 Dec 4 doi: 10.1016/S1470-2045(19)30671-0. PMID: 31812554
Tolerability and activity of ublituximab, umbralisib, and ibrutinib in patients with chronic lymphocytic leukaemia and non-Hodgkin lymphoma: a phase 1 dose escalation and expansion trial.
Nastoupil LJ, Lunning MA, Vose JM, Schreeder MT, Siddiqi T, Flowers CR, Cohen JB, Burger JA, Wierda WG, O'Brien S, Sportelli P, Miskin HP, Purdom MA, Weiss MS, Fowler NH
Lancet Haematol 2019 Feb;6(2):e100-e109. doi: 10.1016/S2352-3026(18)30216-3. PMID: 30709431
Treatment outcome and prognostic factors for primary mediastinal (thymic) B-cell lymphoma: a multicenter study of 106 patients.
Lazzarino M, Orlandi E, Paulli M, Sträter J, Klersy C, Gianelli U, Gargantini L, Rousset MT, Gambacorta M, Marra E, Lavabre-Bertrand T, Magrini U, Manegold C, Bernasconi C, Möller P
J Clin Oncol 1997 Apr;15(4):1646-53. doi: 10.1200/JCO.1997.15.4.1646. PMID: 9193365

Diagnosis

Ibrutinib resistance in a patient with transformed diffuse large B-cell lymphoma from primary pulmonary mucosa-associated lymphoid tissue lymphoma.
Zhou H, Yang L, Dang Q, Huang J, Cheng Y, Zhang Y, Shi W
Cancer Biol Ther 2020 Apr 2;21(4):303-308. Epub 2020 Jan 13 doi: 10.1080/15384047.2019.1700743. PMID: 31931656Free PMC Article
Fatal sclerosing peritonitis associated with primary effusion lymphoma after liver transplantation: a case report.
Testa A, Baiocchini A, Comandini UV, Falasca L, Nardacci R, Maritti M, Loiacono L, Bibbolino C, Rizzi EB, Cristofaro M, Ettorre GM, Vennarecci G, Antonucci G, Del Nonno F
Transplant Proc 2010 Nov;42(9):3849-53. doi: 10.1016/j.transproceed.2010.08.039. PMID: 21094868
Inhibition of phosphatidylinositol 3'-kinase/AKT signaling promotes apoptosis of primary effusion lymphoma cells.
Uddin S, Hussain AR, Al-Hussein KA, Manogaran PS, Wickrema A, Gutierrez MI, Bhatia KG
Clin Cancer Res 2005 Apr 15;11(8):3102-8. doi: 10.1158/1078-0432.CCR-04-1857. PMID: 15837766
Clinical perspectives of primary cardiac lymphoma.
Gowda RM, Khan IA
Angiology 2003 Sep-Oct;54(5):599-604. doi: 10.1177/000331970305400510. PMID: 14565636
Primary cardiac lymphoma in immunocompetent patients: diagnostic and therapeutic management.
Ceresoli GL, Ferreri AJ, Bucci E, Ripa C, Ponzoni M, Villa E
Cancer 1997 Oct 15;80(8):1497-506. doi: 10.1002/(sici)1097-0142(19971015)80:8<1497::aid-cncr18>3.0.co;2-0. PMID: 9338475

Therapy

Daratumumab induces cell-mediated cytotoxicity of primary effusion lymphoma and is active against refractory disease.
Shrestha P, Astter Y, Davis DA, Zhou T, Yuan CM, Ramaswami R, Wang HW, Lurain K, Yarchoan R
Oncoimmunology 2023;12(1):2163784. Epub 2023 Jan 7 doi: 10.1080/2162402X.2022.2163784. PMID: 36632565Free PMC Article
Use of pembrolizumab with or without pomalidomide in HIV-associated non-Hodgkin's lymphoma.
Lurain K, Ramaswami R, Mangusan R, Widell A, Ekwede I, George J, Ambinder R, Cheever M, Gulley JL, Goncalves PH, Wang HW, Uldrick TS, Yarchoan R
J Immunother Cancer 2021 Feb;9(2) doi: 10.1136/jitc-2020-002097. PMID: 33608378Free PMC Article
Ibrutinib resistance in a patient with transformed diffuse large B-cell lymphoma from primary pulmonary mucosa-associated lymphoid tissue lymphoma.
Zhou H, Yang L, Dang Q, Huang J, Cheng Y, Zhang Y, Shi W
Cancer Biol Ther 2020 Apr 2;21(4):303-308. Epub 2020 Jan 13 doi: 10.1080/15384047.2019.1700743. PMID: 31931656Free PMC Article
Pembrolizumab in paediatric patients with advanced melanoma or a PD-L1-positive, advanced, relapsed, or refractory solid tumour or lymphoma (KEYNOTE-051): interim analysis of an open-label, single-arm, phase 1-2 trial.
Geoerger B, Kang HJ, Yalon-Oren M, Marshall LV, Vezina C, Pappo A, Laetsch TW, Petrilli AS, Ebinger M, Toporski J, Glade-Bender J, Nicholls W, Fox E, DuBois SG, Macy ME, Cohn SL, Pathiraja K, Diede SJ, Ebbinghaus S, Pinto N
Lancet Oncol 2020 Jan;21(1):121-133. Epub 2019 Dec 4 doi: 10.1016/S1470-2045(19)30671-0. PMID: 31812554
EBV lymphoproliferative-associated disease and primary cardiac T-cell lymphoma in a STK4 deficient patient: A case report.
Sherkat R, Sabri MR, Dehghan B, Bigdelian H, Reisi N, Afsharmoghadam N, Rahimi H, Rahmanian N, Klein C
Medicine (Baltimore) 2017 Dec;96(48):e8852. doi: 10.1097/MD.0000000000008852. PMID: 29310365Free PMC Article

Prognosis

Ibrutinib resistance in a patient with transformed diffuse large B-cell lymphoma from primary pulmonary mucosa-associated lymphoid tissue lymphoma.
Zhou H, Yang L, Dang Q, Huang J, Cheng Y, Zhang Y, Shi W
Cancer Biol Ther 2020 Apr 2;21(4):303-308. Epub 2020 Jan 13 doi: 10.1080/15384047.2019.1700743. PMID: 31931656Free PMC Article
Pembrolizumab in paediatric patients with advanced melanoma or a PD-L1-positive, advanced, relapsed, or refractory solid tumour or lymphoma (KEYNOTE-051): interim analysis of an open-label, single-arm, phase 1-2 trial.
Geoerger B, Kang HJ, Yalon-Oren M, Marshall LV, Vezina C, Pappo A, Laetsch TW, Petrilli AS, Ebinger M, Toporski J, Glade-Bender J, Nicholls W, Fox E, DuBois SG, Macy ME, Cohn SL, Pathiraja K, Diede SJ, Ebbinghaus S, Pinto N
Lancet Oncol 2020 Jan;21(1):121-133. Epub 2019 Dec 4 doi: 10.1016/S1470-2045(19)30671-0. PMID: 31812554
EBV lymphoproliferative-associated disease and primary cardiac T-cell lymphoma in a STK4 deficient patient: A case report.
Sherkat R, Sabri MR, Dehghan B, Bigdelian H, Reisi N, Afsharmoghadam N, Rahimi H, Rahmanian N, Klein C
Medicine (Baltimore) 2017 Dec;96(48):e8852. doi: 10.1097/MD.0000000000008852. PMID: 29310365Free PMC Article
Fatal sclerosing peritonitis associated with primary effusion lymphoma after liver transplantation: a case report.
Testa A, Baiocchini A, Comandini UV, Falasca L, Nardacci R, Maritti M, Loiacono L, Bibbolino C, Rizzi EB, Cristofaro M, Ettorre GM, Vennarecci G, Antonucci G, Del Nonno F
Transplant Proc 2010 Nov;42(9):3849-53. doi: 10.1016/j.transproceed.2010.08.039. PMID: 21094868
Primary cardiac lymphoma in immunocompetent patients: diagnostic and therapeutic management.
Ceresoli GL, Ferreri AJ, Bucci E, Ripa C, Ponzoni M, Villa E
Cancer 1997 Oct 15;80(8):1497-506. doi: 10.1002/(sici)1097-0142(19971015)80:8<1497::aid-cncr18>3.0.co;2-0. PMID: 9338475

Clinical prediction guides

Pembrolizumab in paediatric patients with advanced melanoma or a PD-L1-positive, advanced, relapsed, or refractory solid tumour or lymphoma (KEYNOTE-051): interim analysis of an open-label, single-arm, phase 1-2 trial.
Geoerger B, Kang HJ, Yalon-Oren M, Marshall LV, Vezina C, Pappo A, Laetsch TW, Petrilli AS, Ebinger M, Toporski J, Glade-Bender J, Nicholls W, Fox E, DuBois SG, Macy ME, Cohn SL, Pathiraja K, Diede SJ, Ebbinghaus S, Pinto N
Lancet Oncol 2020 Jan;21(1):121-133. Epub 2019 Dec 4 doi: 10.1016/S1470-2045(19)30671-0. PMID: 31812554
Evaluation of children with myelodysplastic syndrome: importance of extramedullary disease as a presenting symptom.
Hiçsönmez G, Cetin M, Yenicesu I, Olcay L, Koç A, Aktaş D, Tunçbilek E, Tuncer M
Leuk Lymphoma 2001 Aug;42(4):665-74. doi: 10.3109/10428190109099328. PMID: 11697496
Primary cardiac lymphoma in immunocompetent patients: diagnostic and therapeutic management.
Ceresoli GL, Ferreri AJ, Bucci E, Ripa C, Ponzoni M, Villa E
Cancer 1997 Oct 15;80(8):1497-506. doi: 10.1002/(sici)1097-0142(19971015)80:8<1497::aid-cncr18>3.0.co;2-0. PMID: 9338475
Treatment outcome and prognostic factors for primary mediastinal (thymic) B-cell lymphoma: a multicenter study of 106 patients.
Lazzarino M, Orlandi E, Paulli M, Sträter J, Klersy C, Gianelli U, Gargantini L, Rousset MT, Gambacorta M, Marra E, Lavabre-Bertrand T, Magrini U, Manegold C, Bernasconi C, Möller P
J Clin Oncol 1997 Apr;15(4):1646-53. doi: 10.1200/JCO.1997.15.4.1646. PMID: 9193365

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