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Angioimmunoblastic T-cell lymphoma

MedGen UID:
7025
Concept ID:
C0020981
Neoplastic Process
Synonyms: Angioimmunoblastic Lymphadenopathies; Angioimmunoblastic Lymphadenopathy; Immunoblastic Lymphadenopathies; Immunoblastic Lymphadenopathy; Lymphadenopathies, Angioimmunoblastic; Lymphadenopathies, Immunoblastic; Lymphadenopathy, Angioimmunoblastic; Lymphadenopathy, Immunoblastic
SNOMED CT: Peripheral T-cell lymphoma, AILD (Angioimmunoblastic Lymphadenopathy with Dysproteinemia) (835009); Angioimmunoblastic lymphoma (835009); Angioimmunoblastic T-cell lymphoma (835009); Angioimmunoblastic T-cell lymphoma (413537009); Angioimmunoblastic lymphadenopathy (52097008)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0004977
Orphanet: ORPHA86886

Definition

A rare T-cell non-Hodgkin lymphoma characterized by infiltration of lymph nodes by neoplastic cells of T follicular helper cell origin with a polymorphous inflammatory background including markedly increased follicular dendritic cells and EBV-positive B-cells, as well as prominent proliferation of high endothelial venules. The spleen, liver, skin, and bone marrow are also frequently involved. Patients typically present with generalized lymphadenopathy, hepatosplenomegaly, systemic symptoms, and polyclonal hypergammaglobulinemia. Pruritic skin rash, arthritis, pleural effusion, and ascites may also be observed. The condition is aggressive with generally poor prognosis. [from ORDO]

Professional guidelines

PubMed

Horwitz SM, Ansell S, Ai WZ, Barnes J, Barta SK, Brammer J, Clemens MW, Dogan A, Foss F, Ghione P, Goodman AM, Guitart J, Halwani A, Haverkos BM, Hoppe RT, Jacobsen E, Jagadeesh D, Jones A, Kallam A, Kim YH, Kumar K, Mehta-Shah N, Olsen EA, Rajguru SA, Rozati S, Said J, Shaver A, Shea L, Shinohara MM, Sokol L, Torres-Cabala C, Wilcox R, Wu P, Zain J, Dwyer M, Sundar H
J Natl Compr Canc Netw 2022 Mar;20(3):285-308. doi: 10.6004/jnccn.2022.0015. PMID: 35276674
Vega F, Amador C, Chadburn A, Feldman AL, Hsi ED, Wang W, Medeiros LJ
Ann Diagn Pathol 2020 Dec;49:151623. Epub 2020 Sep 13 doi: 10.1016/j.anndiagpath.2020.151623. PMID: 32947231
Mosalpuria K, Bociek RG, Vose JM
Semin Hematol 2014 Jan;51(1):52-8. Epub 2013 Nov 14 doi: 10.1053/j.seminhematol.2013.11.008. PMID: 24468316

Recent clinical studies

Etiology

Chen JJ, Tokumori FC, Del Guzzo C, Kim J, Ruan J
Curr Hematol Malig Rep 2024 Jun;19(3):93-103. Epub 2024 Mar 7 doi: 10.1007/s11899-024-00727-w. PMID: 38451372
Horwitz SM, Ansell S, Ai WZ, Barnes J, Barta SK, Brammer J, Clemens MW, Dogan A, Foss F, Ghione P, Goodman AM, Guitart J, Halwani A, Haverkos BM, Hoppe RT, Jacobsen E, Jagadeesh D, Jones A, Kallam A, Kim YH, Kumar K, Mehta-Shah N, Olsen EA, Rajguru SA, Rozati S, Said J, Shaver A, Shea L, Shinohara MM, Sokol L, Torres-Cabala C, Wilcox R, Wu P, Zain J, Dwyer M, Sundar H
J Natl Compr Canc Netw 2022 Mar;20(3):285-308. doi: 10.6004/jnccn.2022.0015. PMID: 35276674
Advani RH, Skrypets T, Civallero M, Spinner MA, Manni M, Kim WS, Shustov AR, Horwitz SM, Hitz F, Cabrera ME, Dlouhy I, Vassallo J, Pileri SA, Inghirami G, Montoto S, Vitolo U, Radford J, Vose JM, Federico M
Blood 2021 Jul 22;138(3):213-220. doi: 10.1182/blood.2020010387. PMID: 34292324Free PMC Article
Stoll JR, Willner J, Oh Y, Pulitzer M, Moskowitz A, Horwitz S, Myskowski P, Noor SJ
J Am Acad Dermatol 2021 Nov;85(5):1073-1090. Epub 2021 Apr 30 doi: 10.1016/j.jaad.2021.04.080. PMID: 33940098
Dogan A, Attygalle AD, Kyriakou C
Br J Haematol 2003 Jun;121(5):681-91. doi: 10.1046/j.1365-2141.2003.04335.x. PMID: 12780782

Diagnosis

Horwitz SM, Ansell S, Ai WZ, Barnes J, Barta SK, Brammer J, Clemens MW, Dogan A, Foss F, Ghione P, Goodman AM, Guitart J, Halwani A, Haverkos BM, Hoppe RT, Jacobsen E, Jagadeesh D, Jones A, Kallam A, Kim YH, Kumar K, Mehta-Shah N, Olsen EA, Rajguru SA, Rozati S, Said J, Shaver A, Shea L, Shinohara MM, Sokol L, Torres-Cabala C, Wilcox R, Wu P, Zain J, Dwyer M, Sundar H
J Natl Compr Canc Netw 2022 Mar;20(3):285-308. doi: 10.6004/jnccn.2022.0015. PMID: 35276674
Mohammed Saleh MF, Kotb A, Abdallah GEM, Muhsen IN, El Fakih R, Aljurf M
Curr Oncol 2021 Dec 20;28(6):5480-5498. doi: 10.3390/curroncol28060456. PMID: 34940095Free PMC Article
Xie Y, Jaffe ES
Am J Clin Pathol 2021 Jun 17;156(1):1-14. doi: 10.1093/ajcp/aqab090. PMID: 34117736Free PMC Article
Jiang M, Bennani NN, Feldman AL
Expert Rev Hematol 2017 Mar;10(3):239-249. Epub 2017 Jan 29 doi: 10.1080/17474086.2017.1281122. PMID: 28133975Free PMC Article
Lunning MA, Vose JM
Blood 2017 Mar 2;129(9):1095-1102. Epub 2017 Jan 23 doi: 10.1182/blood-2016-09-692541. PMID: 28115369

Therapy

Advani RH, Skrypets T, Civallero M, Spinner MA, Manni M, Kim WS, Shustov AR, Horwitz SM, Hitz F, Cabrera ME, Dlouhy I, Vassallo J, Pileri SA, Inghirami G, Montoto S, Vitolo U, Radford J, Vose JM, Federico M
Blood 2021 Jul 22;138(3):213-220. doi: 10.1182/blood.2020010387. PMID: 34292324Free PMC Article
Cogan JC, Liu Y, Amengual JE
Curr Treat Options Oncol 2020 Jun 29;21(8):61. doi: 10.1007/s11864-020-00761-9. PMID: 32601883
Lunning MA, Vose JM
Blood 2017 Mar 2;129(9):1095-1102. Epub 2017 Jan 23 doi: 10.1182/blood-2016-09-692541. PMID: 28115369
Mosalpuria K, Bociek RG, Vose JM
Semin Hematol 2014 Jan;51(1):52-8. Epub 2013 Nov 14 doi: 10.1053/j.seminhematol.2013.11.008. PMID: 24468316
Iannitto E, Ferreri AJ, Minardi V, Tripodo C, Kreipe HH
Crit Rev Oncol Hematol 2008 Dec;68(3):264-71. Epub 2008 Aug 6 doi: 10.1016/j.critrevonc.2008.06.012. PMID: 18684638

Prognosis

Chen JJ, Tokumori FC, Del Guzzo C, Kim J, Ruan J
Curr Hematol Malig Rep 2024 Jun;19(3):93-103. Epub 2024 Mar 7 doi: 10.1007/s11899-024-00727-w. PMID: 38451372
Zhang Q, Yin L, Lai Q, Zhao Y, Peng H
Clin Exp Med 2023 Dec;23(8):4219-4235. Epub 2023 Sep 27 doi: 10.1007/s10238-023-01197-9. PMID: 37759042
Mohammed Saleh MF, Kotb A, Abdallah GEM, Muhsen IN, El Fakih R, Aljurf M
Curr Oncol 2021 Dec 20;28(6):5480-5498. doi: 10.3390/curroncol28060456. PMID: 34940095Free PMC Article
Chiba S, Sakata-Yanagimoto M
Leukemia 2020 Oct;34(10):2592-2606. Epub 2020 Jul 23 doi: 10.1038/s41375-020-0990-y. PMID: 32704161Free PMC Article
Jiang M, Bennani NN, Feldman AL
Expert Rev Hematol 2017 Mar;10(3):239-249. Epub 2017 Jan 29 doi: 10.1080/17474086.2017.1281122. PMID: 28133975Free PMC Article

Clinical prediction guides

Rai S, Kim WS, Ando K, Choi I, Izutsu K, Tsukamoto N, Yokoyama M, Tsukasaki K, Kuroda J, Ando J, Hidaka M, Koh Y, Shibayama H, Uchida T, Yang DH, Ishitsuka K, Ishizawa K, Kim JS, Lee HG, Minami H, Eom HS, Kurosawa M, Lee JH, Lee JS, Lee WS, Nagai H, Shindo T, Yoon DH, Yoshida S, Gillings M, Onogi H, Tobinai K
Haematologica 2023 Mar 1;108(3):811-821. doi: 10.3324/haematol.2022.280996. PMID: 36200417Free PMC Article
Advani RH, Skrypets T, Civallero M, Spinner MA, Manni M, Kim WS, Shustov AR, Horwitz SM, Hitz F, Cabrera ME, Dlouhy I, Vassallo J, Pileri SA, Inghirami G, Montoto S, Vitolo U, Radford J, Vose JM, Federico M
Blood 2021 Jul 22;138(3):213-220. doi: 10.1182/blood.2020010387. PMID: 34292324Free PMC Article
Tari G, Lemonnier F, Morschhauser F
Curr Opin Oncol 2021 Sep 1;33(5):400-405. doi: 10.1097/CCO.0000000000000773. PMID: 34230442
Horwitz SM, Advani RH, Bartlett NL, Jacobsen ED, Sharman JP, O'Connor OA, Siddiqi T, Kennedy DA, Oki Y
Blood 2014 May 15;123(20):3095-100. Epub 2014 Mar 20 doi: 10.1182/blood-2013-12-542142. PMID: 24652992Free PMC Article
Iqbal J, Wright G, Wang C, Rosenwald A, Gascoyne RD, Weisenburger DD, Greiner TC, Smith L, Guo S, Wilcox RA, Teh BT, Lim ST, Tan SY, Rimsza LM, Jaffe ES, Campo E, Martinez A, Delabie J, Braziel RM, Cook JR, Tubbs RR, Ott G, Geissinger E, Gaulard P, Piccaluga PP, Pileri SA, Au WY, Nakamura S, Seto M, Berger F, de Leval L, Connors JM, Armitage J, Vose J, Chan WC, Staudt LM; Lymphoma Leukemia Molecular Profiling Project and the International Peripheral T-cell Lymphoma Project
Blood 2014 May 8;123(19):2915-23. Epub 2014 Mar 14 doi: 10.1182/blood-2013-11-536359. PMID: 24632715Free PMC Article

Recent systematic reviews

Munoz de Toro M, Fernandez-Pol S
J Clin Pathol 2024 Nov 19;77(12):802-809. doi: 10.1136/jcp-2024-209513. PMID: 39366734
Nishimura MF, Takahashi T, Takaoka K, Macapagal S, Wannaphut C, Nishikori A, Toda H, Nishimura Y, Sato Y
J Clin Exp Hematop 2024;64(2):97-106. doi: 10.3960/jslrt.24007. PMID: 38925977Free PMC Article
Zhai Y, Wang J, Jiang Y, Wu W, Lv Y, Xu H, Tian L, Sun H, Zhao Z, Li L
Expert Rev Hematol 2022 Mar;15(3):265-272. Epub 2022 Feb 21 doi: 10.1080/17474086.2022.2042247. PMID: 35152814
Nguyen PN, Tran NTB, Nguyen TPX, Ngo TNM, Lai DV, Deel CD, Hassell LA, Vuong HG
Clin Lymphoma Myeloma Leuk 2021 Jul;21(7):431-438. Epub 2021 Mar 19 doi: 10.1016/j.clml.2021.03.002. PMID: 33849798

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