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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.
See: This record
Not genetically inherited (Orphanet)
 
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

Current Treatment of Peripheral T-cell Lymphoma.
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
T-Cell Lymphomas, Version 2.2022, NCCN Clinical Practice Guidelines in Oncology.
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
Angioimmunoblastic T-cell lymphoma management.
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

T-Cell Lymphomas, Version 2.2022, NCCN Clinical Practice Guidelines in Oncology.
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
Primary cutaneous T-cell lymphomas other than mycosis fungoides and Sézary syndrome. Part I: Clinical and histologic features and diagnosis.
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
A suggested immunohistochemical algorithm for the classification of T-cell lymphomas involving lymph nodes.
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
Genetic drivers of oncogenic pathways in molecular subgroups of peripheral T-cell lymphoma.
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
Gene expression signatures delineate biological and prognostic subgroups in peripheral T-cell lymphoma.
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

T-Cell Lymphomas, Version 2.2022, NCCN Clinical Practice Guidelines in Oncology.
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
Recent Advances in Diagnosis and Therapy of Angioimmunoblastic T Cell Lymphoma.
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
How I Diagnose Angioimmunoblastic T-Cell Lymphoma.
Xie Y, Jaffe ES
Am J Clin Pathol 2021 Jun 17;156(1):1-14. doi: 10.1093/ajcp/aqab090. PMID: 34117736Free PMC Article
Lymphoma classification update: T-cell lymphomas, Hodgkin lymphomas, and histiocytic/dendritic cell neoplasms.
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
Angioimmunoblastic T-cell lymphoma: the many-faced lymphoma.
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

Darinaparsin in patients with relapsed or refractory peripheral T-cell lymphoma: results of an Asian phase 2 study.
Kim WS, Fukuhara N, Yoon DH, Yamamoto K, Uchida T, Negoro E, Izutsu K, Terui Y, Nakajima H, Ando K, Suehiro Y, Kang HJ, Ko PS, Nagahama F, Sonehara Y, Nagai H, Tien HF, Kwong YL, Tobinai K
Blood Adv 2023 Sep 12;7(17):4903-4912. doi: 10.1182/bloodadvances.2022008615. PMID: 36661315Free PMC Article
Oral HDAC inhibitor tucidinostat in patients with relapsed or refractory peripheral T-cell lymphoma: phase IIb results.
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
Outcomes and prognostic factors in angioimmunoblastic T-cell lymphoma: final report from the international T-cell Project.
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
Angioimmunoblastic T-cell lymphoma: the many-faced lymphoma.
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
Angioimmunoblastic T-cell lymphoma.
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

Recent Advances in Diagnosis and Therapy of Angioimmunoblastic T Cell Lymphoma.
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
Primary cutaneous T-cell lymphomas other than mycosis fungoides and Sézary syndrome. Part I: Clinical and histologic features and diagnosis.
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
Advances in understanding of angioimmunoblastic T-cell lymphoma.
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
Genetic drivers of oncogenic pathways in molecular subgroups of peripheral T-cell lymphoma.
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
Lymphoma classification update: T-cell lymphomas, Hodgkin lymphomas, and histiocytic/dendritic cell neoplasms.
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

Darinaparsin in patients with relapsed or refractory peripheral T-cell lymphoma: results of an Asian phase 2 study.
Kim WS, Fukuhara N, Yoon DH, Yamamoto K, Uchida T, Negoro E, Izutsu K, Terui Y, Nakajima H, Ando K, Suehiro Y, Kang HJ, Ko PS, Nagahama F, Sonehara Y, Nagai H, Tien HF, Kwong YL, Tobinai K
Blood Adv 2023 Sep 12;7(17):4903-4912. doi: 10.1182/bloodadvances.2022008615. PMID: 36661315Free PMC Article
Oral HDAC inhibitor tucidinostat in patients with relapsed or refractory peripheral T-cell lymphoma: phase IIb results.
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
Outcomes and prognostic factors in angioimmunoblastic T-cell lymphoma: final report from the international T-cell Project.
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
Objective responses in relapsed T-cell lymphomas with single-agent brentuximab vedotin.
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
Gene expression signatures delineate biological and prognostic subgroups in peripheral T-cell lymphoma.
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

The efficiency of autologous stem cell transplantation as the first-line treatment for nodal peripheral T-cell lymphoma: results of a systematic review and meta-analysis.
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
Clinicopathological Implications of RHOA Mutations in Angioimmunoblastic T-Cell Lymphoma: A Meta-analysis: RHOA mutations in AITL.
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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