Angioimmunoblastic T‑cell lymphoma: case report demonstrating the utility of molecular mutational analysis
DOI:
https://doi.org/10.5281/zenodo.16789547Keywords:
T-Cell lymphoma, peripheral T‑cell lymphoma, molecular pathologyAbstract
Angioimmunoblastic T-cell lymphoma (AITL) is a distinctive, yet rare, peripheral T-cell lymphoma. In the United States, the incidence is approximately 0.05 cases per 100,000 person-years, representing 15% to 20% of peripheral T-cell lymphomas and 1% to 2% of non-Hodgkin lymphomas. A 62-year-old man with systemic symptoms and diffuse lymphadenopathy was presented. The neoplastic cells lacked CD10/BCL6, but showed strong expression of PD-1, ICOS and CXCL13, and harbored mutations in TET2 and DNMT3A, confirming a follicular helper T-cell (TFH) phenotype. Expanded TFH immunostaining, combined with targeted mutational analysis, can resolve diagnostically ambiguous AITL presentations.
Downloads
References
Morton LM, Wang SS, Devesa SS, Hartge P, Weisenburger DD, Linet MS. Lymphoma incidence patterns by WHO subtype in the United States, 1992-2001. Blood [Internet]. 2006 [cited 21 May 2025]; 107(1):265-76. DOI: https://doi.org/10.1182/blood-2005-06-2508
De Leval L, Parrens M, Le Bras F, Jais JP, Fataccioli V, Martin A, et al. Angioimmunoblastic T-cell lymphoma is the most common T-cell lymphoma in two distinct French information data sets. Haematologica [Internet]. 2015 [cited 21 May 2025]; 100(9). DOI: https://doi.org/10.3324/haematol.2015.126300
Zeng H, Zheng R, Guo Y, Zhang S, Zou X, Wang N, et al. Cancer survival in China, 2003-2005: a population-based study. Int J Cancer [Internet]. 2015 [cited 21 May 2025];136(8):1921-1930. DOI: https://doi.org/10.1002/ijc.29227
Federico M, Rudiger T, Bellei M, Nathwani BN, Luminari S, Coiffier B, et al. Clinicopathologic characteristics of angioimmunoblastic T-cell lymphoma: analysis of the international peripheral T-cell lymphoma project. J Clin Oncol [Internet]. 2013 [cited 21 May 2025]; 31(2):240-246. DOI: https://doi.org/10.1200/JCO.2011.37.3647
Xie Y, Jaffe ES. How I Diagnose Angioimmunoblastic T-Cell Lymphoma. Am J Clin Pathol [Internet]. 2021 [cited 21 May 2025]; 156(1):1-14. DOI: https://doi.org/10.1093/ajcp/aqab090
Attygalle AD, Chuang SS, Diss TC, Du MQ, Isaacson PG, Dogan A. Distinguishing angioimmunoblastic T-cell lymphoma from peripheral T-cell lymphoma, unspecified, using morphology, immunophenotype and molecular genetics. Histopathology [Internet]. 2007 [cited 21 May 2025]; 50(4):498-508. DOI: https://doi.org/10.1111/j.1365-2559.2007.02632.x
Savage KJ, Horwitz SM, Advani R, Christensen JH, Domingo-Domenech E, Rossi G, et al. Role of stem cell transplant in CD30+ PTCL following frontline brentuximab vedotin plus CHP or CHOP in ECHELON-2. Blood Adv [Internet]. 2022 [cited 21 May 2025]; 6(19):5550-5555. DOI: https://doi.org/10.1182/bloodadvances.2020003971
O'Connor OA, Horwitz S, Masszi T, Van Hoof A, Brown P, Doorduijn J, et al. Belinostat in patients with relapsed or refractory peripheral T-Cell lymphoma: Results of the pivotal phase II BELIEF (CLN-19) Study. J Clin Oncol [Internet]. 2015 [cited 21 May 2025]; 33(23):2492-2499. DOI: https://doi.org/10.1200/jco.2014.59.2782
Lemonnier F, Dupuis J, Sujobert P, Tournillhac O, Cheminant M, Sarkozy C, et al. Treatment with 5-azacytidine induces a sustained response in patients with angioimmunoblastic T-cell lymphoma. Blood [Internet]. 2018 [cited 21 May 2025]; 132(21):2305-2309. DOI: https://doi.org/10.1182/blood-2018-04-840538
Horwitz S, O'Connor OA, Pro B, Illidge T, Fanale M, Advani R, et al. Brentuximab vedotin with chemotherapy for CD30-positive peripheral T-cell lymphoma (ECHELON-2): a global, double-blind, randomised, phase 3 trial. Lancet [Internet]. 2019 [cited 21 May 2025]; 393(10168):229-240. DOI: https://doi.org/10.1016/s0140-6736(18)32984-2
Published
How to Cite
Issue
Section
License
Copyright (c) 2025 Alvaro Frometa, Robert W. Allan, Yasna Chaudary
This work is licensed under a Creative Commons Attribution 4.0 International License.
