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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

Stuver R, Epstein-Peterson ZD, Johnson WT, Khan N, Lewis N, Moskowitz AJ, Sauter CS, Horwitz S
Oncology (Williston Park) 2022 May 9;36(5):293-305. doi: 10.46883/2022.25920960. PMID: 35576176
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
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

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
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
Vega F, Medeiros LJ
Hum Pathol 2020 Aug;102:104-116. Epub 2020 May 29 doi: 10.1016/j.humpath.2020.05.006. PMID: 32479842
Heavican TB, Bouska A, Yu J, Lone W, Amador C, Gong Q, Zhang W, Li Y, Dave BJ, Nairismägi ML, Greiner TC, Vose J, Weisenburger DD, Lachel C, Wang C, Fu K, Stevens JM, Lim ST, Ong CK, Gascoyne RD, Missiaglia E, Lemonnier F, Haioun C, Hartmann S, Pedersen MB, Laginestra MA, Wilcox RA, Teh BT, Yoshida N, Ohshima K, Seto M, Rosenwald A, Ott G, Campo E, Rimsza LM, Jaffe ES, Braziel RM, d'Amore F, Inghirami G, Bertoni F, de Leval L, Gaulard P, Staudt LM, McKeithan TW, Pileri S, Chan WC, Iqbal J
Blood 2019 Apr 11;133(15):1664-1676. Epub 2019 Feb 19 doi: 10.1182/blood-2018-09-872549. PMID: 30782609Free 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

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

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
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
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
Schmitz N, Trümper L, Ziepert M, Nickelsen M, Ho AD, Metzner B, Peter N, Loeffler M, Rosenwald A, Pfreundschuh M
Blood 2010 Nov 4;116(18):3418-25. Epub 2010 Jul 21 doi: 10.1182/blood-2010-02-270785. PMID: 20660290

Prognosis

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
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
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
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

Clinical prediction guides

Zhang C, Mou B, Xu J, Wang J, Liu Q, Yang Y, Tang W, Zhong X, Xu C
Br J Haematol 2023 Dec;203(5):807-819. Epub 2023 Aug 30 doi: 10.1111/bjh.19089. PMID: 37646306
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
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

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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