{"entity":{"id":"angioimmunoblastic-t-cell-lymphoma","kind":"cancer","name":"Nodal T-follicular helper cell lymphoma, angioimmunoblastic type (angioimmunoblastic T-cell lymphoma)","aka":["Angioimmunoblastic T-cell lymphoma","AITL","Nodal TFH lymphoma","Nodal TFH lymphoma (angioimmunoblastic type)","nTFHL-AI","Angioimmunoblastic lymphadenopathy with dysproteinaemia"],"tldr":"Angioimmunoblastic T-cell lymphoma, now called nodal T-follicular helper cell lymphoma of angioimmunoblastic type, is one of the commonest T-cell lymphomas and mostly affects people over 60. It presents with widespread swollen nodes, fever, rash and immune upsets such as anaemia; about four in ten people are alive five years after chemotherapy, more after a transplant in first remission.","summary":"WHO-HAEM5 groups angioimmunoblastic T-cell lymphoma with follicular and not-otherwise-specified T-follicular helper lymphomas as nodal T-follicular helper cell lymphoma, angioimmunoblastic type being the commonest, defined by TFH markers (PD1, CXCL13, ICOS, BCL6, CD10), a polymorphous infiltrate with arborising venules and expanded follicular dendritic cell meshworks, EBV-positive B cells, and recurrent TET2, DNMT3A, RHOA G17V and IDH2 R172 mutations (Alaggio 2022). In the International Project, 76 percent presented with generalised lymphadenopathy and 89 percent with stage III or IV disease; rash occurred in 21 percent, haemolytic anaemia in 13 percent and hypergammaglobulinaemia in 30 percent; five-year overall and failure-free survival were 33 and 18 percent (Federico 2013). In the prospective T-cell Project, 282 patients had a median age of 64, 81 percent received anthracycline-based regimens, 13 percent had autologous transplant in first complete remission with improved outcomes, five-year overall and progression-free survival were 44 and 32 percent, and age 60 or over, poor performance status, raised C-reactive protein and raised beta-2 microglobulin predicted worse outcome (Advani 2021).\n\nHow it differs from its parent: the peripheral T-cell lymphoma page covers the group; this type is defined by its TFH origin and epigenetic mutations, which make it the T-cell lymphoma most responsive to histone deacetylase inhibitors and hypomethylating agents, and by its immune manifestations (autoimmune haemolysis, polyclonal hypergammaglobulinaemia, rashes) that can precede the diagnosis.\n\nHow common: 18.5 percent of peripheral T-cell lymphomas (Federico 2013).\n\nTreatment: CHOP-based chemotherapy (with etoposide in younger patients) and autologous transplant in first remission for fit patients, as on the parent page; romidepsin, belinostat, pralatrexate, and azacitidine with or without romidepsin in relapsed disease, with the TFH-phenotype trial of duvelisib (TERZO) linked here; brentuximab vedotin when CD30 is expressed (Advani 2021 for the transplant data).","asOf":"2026-09-24","wikipedia":"https://en.wikipedia.org/wiki/Angioimmunoblastic_T-cell_lymphoma","links":[{"label":"NCI PDQ: adult non-Hodgkin lymphoma treatment","url":"https://www.cancer.gov/types/lymphoma/patient/adult-nhl-treatment-pdq"},{"label":"Alaggio 2022, Leukemia: the 5th edition WHO classification of haematolymphoid tumours, lymphoid neoplasms","url":"https://doi.org/10.1038/s41375-022-01620-2"},{"label":"Federico 2013, JCO: angioimmunoblastic T-cell lymphoma in the International Peripheral T-cell Lymphoma Project","url":"https://doi.org/10.1200/jco.2011.37.3647"},{"label":"Advani 2021, Blood: outcomes and prognostic factors in angioimmunoblastic T-cell lymphoma, the international T-cell Project","url":"https://doi.org/10.1182/blood.2020010387"}],"tags":["subtype-page","wave4","haematologic","rare"],"related":["peripheral-t-cell-lymphoma","hepatosplenic-t-cell-lymphoma","cutaneous-t-cell-lymphoma","dlbcl"],"cancers":[],"sections":[],"technologies":[],"targets":[],"drugs":["cyclophosphamide","romidepsin","belinostat","pralatrexate","azacitidine","brentuximab-vedotin"],"companies":[],"institutions":[],"pathways":[],"terms":["r-chop"],"trials":["nct06522737"],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"group":"haematologic","burden":"18.5 percent of peripheral T-cell and NK-cell lymphomas: 243 of 1,314 patients in the International Peripheral T-cell Lymphoma Project, making it one of the two commonest types (Federico 2013).","subtypes":["Nodal TFH lymphoma, angioimmunoblastic type (the commonest)","Nodal TFH lymphoma, follicular type","Nodal TFH lymphoma, not otherwise specified","Angioimmunoblastic T-cell lymphoma with EBV-positive B-cell proliferation or secondary B-cell lymphoma"],"biomarkers":["TFH markers: PD1, CXCL13, ICOS, BCL6, CD10","TET2, DNMT3A, RHOA G17V and IDH2 R172 mutations","EBV-positive B immunoblasts","C-reactive protein and beta-2 microglobulin (AITL prognostic score)"],"standardOfCare":[{"setting":"First line","approach":"CHOP-based chemotherapy, with etoposide in younger patients, and autologous transplant in first complete remission for fit patients (T-cell Project data).","refs":["peripheral-t-cell-lymphoma","cyclophosphamide","r-chop"]},{"setting":"Relapsed","approach":"Romidepsin, belinostat or pralatrexate; azacitidine with romidepsin; duvelisib in the TFH-phenotype trial TERZO; brentuximab vedotin when CD30-positive.","refs":["romidepsin","belinostat","pralatrexate","azacitidine","brentuximab-vedotin","nct06522737"]}],"stateOfArt":[],"history":[],"pipeline":[],"openProblems":[],"parent":"peripheral-t-cell-lymphoma"},"route":"/cancers/angioimmunoblastic-t-cell-lymphoma/","neighbours":{"cancer":[{"id":"cutaneous-t-cell-lymphoma","kind":"cancer","name":"Cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome)","route":"/cancers/cutaneous-t-cell-lymphoma/"},{"id":"dlbcl","kind":"cancer","name":"Diffuse large B-cell lymphoma","route":"/cancers/dlbcl/"},{"id":"hepatosplenic-t-cell-lymphoma","kind":"cancer","name":"Hepatosplenic T-cell lymphoma","route":"/cancers/hepatosplenic-t-cell-lymphoma/"},{"id":"peripheral-t-cell-lymphoma","kind":"cancer","name":"Peripheral T-cell lymphomas (including cutaneous T-cell lymphoma)","route":"/cancers/peripheral-t-cell-lymphoma/"}],"drug":[{"id":"azacitidine","kind":"drug","name":"Azacitidine","route":"/drugs/azacitidine/"},{"id":"belinostat","kind":"drug","name":"Belinostat","route":"/drugs/belinostat/"},{"id":"brentuximab-vedotin","kind":"drug","name":"Brentuximab vedotin","route":"/drugs/brentuximab-vedotin/"},{"id":"cyclophosphamide","kind":"drug","name":"Cyclophosphamide","route":"/drugs/cyclophosphamide/"},{"id":"pralatrexate","kind":"drug","name":"Pralatrexate","route":"/drugs/pralatrexate/"},{"id":"romidepsin","kind":"drug","name":"Romidepsin","route":"/drugs/romidepsin/"}],"term":[{"id":"r-chop","kind":"term","name":"R-CHOP (lymphoma chemoimmunotherapy)","route":"/terms/r-chop/"}],"trial":[{"id":"nct06522737","kind":"trial","name":"A Study of Duvelisib Versus Gemcitabine or Bendamustine in Participants With Relapsed/Refractory Nodal T Cell Lymphoma With T Follicular Helper (TFH) Phenotype","route":"/trials/nct06522737/"}]}}