# Nodal T-follicular helper cell lymphoma, angioimmunoblastic type (angioimmunoblastic T-cell lymphoma)

Source: https://onco.cc/cancers/angioimmunoblastic-t-cell-lymphoma/  
OnCo record `angioimmunoblastic-t-cell-lymphoma` (Cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

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).

How 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.

How common: 18.5 percent of peripheral T-cell lymphomas (Federico 2013).

Treatment: 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).

## Fields

- Kind: Cancer
- Last checked: 2026-09-24
- Also known as: Angioimmunoblastic T-cell lymphoma; AITL; Nodal TFH lymphoma; Nodal TFH lymphoma (angioimmunoblastic type); nTFHL-AI; Angioimmunoblastic lymphadenopathy with dysproteinaemia
- Tags: subtype-page; wave4; haematologic; rare
- 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)

## Sections of this record

The page is a hub with ten sections in reading order; large sections have their own page. The same plan as JSON: https://onco.cc/api/v1/cancers/angioimmunoblastic-t-cell-lymphoma/sections.json

- Overview (on the hub): The TL;DR, the family this cancer belongs to, the organ, who gets it and what the state of the art is. https://onco.cc/cancers/angioimmunoblastic-t-cell-lymphoma/#overview [3 state-of-the-art points]
- Types and stages (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/angioimmunoblastic-t-cell-lymphoma/#what-it-is [4 subtypes]
- Symptoms and diagnosis (on the hub): How this cancer shows itself, how the diagnosis is confirmed, and the biomarkers clinicians test for. https://onco.cc/cancers/angioimmunoblastic-t-cell-lymphoma/#finding-it [4 biomarkers]
- Treatment (on the hub): The standard of care by setting, the medicines, surgery and radiotherapy named in it, and the regimens behind them. https://onco.cc/cancers/angioimmunoblastic-t-cell-lymphoma/#treating-it [3 settings, 2 decisions with options]
- Trials and papers (on the hub): Trials recruiting now, the landmark trials, the trials held by this cancer's subtypes, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/angioimmunoblastic-t-cell-lymphoma/#evidence [1 trial]
- Biology and targets (on the hub): The molecular landscape: the targets and how often each appears, the pathways, the mechanics stages and the preclinical models. https://onco.cc/cancers/angioimmunoblastic-t-cell-lymphoma/#science [9 targets, 3 pathways]
- Countries and centres (own page): Cases by country, the UK and NHS pathway and other country lenses, the expert centres with trials on record, and the centres named on this cancer's subtypes. https://onco.cc/cancers/angioimmunoblastic-t-cell-lymphoma/where-you-are/ [25 UK centres]
- Decisions and support (on the hub): The decisions you may face, the aids that walk through them, the warnings on record, the first sixty days and the questions to ask. https://onco.cc/cancers/angioimmunoblastic-t-cell-lymphoma/#living-with-it [13 questions, 6 red cards]
- Pipeline and open problems (own page): Everything in development, the medicines held by this cancer's subtypes, the open problems and what is being done about them, the roadmaps, and what changed on this record. https://onco.cc/cancers/angioimmunoblastic-t-cell-lymphoma/coming/ [10 medicines, 1 idea]
- Data (own page): Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked. https://onco.cc/cancers/angioimmunoblastic-t-cell-lymphoma/data/ [64 connected records]

## Standard of care

- First line: CHOP-based chemotherapy, with etoposide in younger patients, and autologous transplant in first complete remission for fit patients (T-cell Project data). ([Peripheral T-cell lymphomas (including cutaneous T-cell lymphoma)](https://onco.cc/cancers/peripheral-t-cell-lymphoma/), [Cyclophosphamide](https://onco.cc/drugs/cyclophosphamide/), [R-CHOP (lymphoma chemoimmunotherapy)](https://onco.cc/terms/r-chop/))
- Relapsed: Romidepsin, belinostat or pralatrexate; azacitidine with romidepsin; duvelisib in the TFH-phenotype trial TERZO; brentuximab vedotin when CD30-positive. ([Romidepsin](https://onco.cc/drugs/romidepsin/), [Belinostat](https://onco.cc/drugs/belinostat/), [Pralatrexate](https://onco.cc/drugs/pralatrexate/), [Azacitidine](https://onco.cc/drugs/azacitidine/), [Brentuximab vedotin](https://onco.cc/drugs/brentuximab-vedotin/), [A Study of Duvelisib Versus Gemcitabine or Bendamustine in Participants With Relapsed/Refractory Nodal T Cell Lymphoma With T Follicular Helper (TFH) Phenotype](https://onco.cc/trials/nct06522737/))
- Angioimmunoblastic T-cell lymphoma: treated as a nodal peripheral T-cell lymphoma, with two differences: First line is CHOP or CHOEP, with brentuximab vedotin substituted for vincristine where the tumour expresses CD30, and autologous transplant consolidation of a first remission in patients fit for it, exactly as for other nodal T-cell lymphomas. Two things are specific.

The presentation is often autoimmune rather than oncological: rash, polyclonal hypergammaglobulinaemia, autoimmune haemolytic anaemia, arthritis and a positive Coombs test in a systemically unwell older person. Corticosteroids produce a rapid response that can be mistaken for a diagnosis, and the lymphoma returns as soon as they are reduced. Infection risk is high because the immune system is already disordered, so prophylaxis against Pneumocystis and herpes is given from the start.

The mutational profile, TET2, DNMT3A, IDH2 and RHOA G17V, is the same set that produces clonal haematopoiesis, and it is the reason hypomethylating agents such as azacitidine and histone deacetylase inhibitors have more activity here than in other T-cell lymphomas. They are not yet a standard first-line option and belong in a trial. ([The lymphoma regimen alphabet: R-CHOP, pola-R-CHP, DA-EPOCH-R, ABVD, BEACOPP and the rest](https://onco.cc/terms/lymphoma-tx-regimen-alphabet/), [Brentuximab vedotin](https://onco.cc/drugs/brentuximab-vedotin/), [Cyclophosphamide](https://onco.cc/drugs/cyclophosphamide/), [Doxorubicin](https://onco.cc/drugs/doxorubicin/), [Vincristine](https://onco.cc/drugs/vincristine/), [Prednisone](https://onco.cc/drugs/prednisone/), [Etoposide](https://onco.cc/drugs/etoposide/), [Romidepsin](https://onco.cc/drugs/romidepsin/), [Belinostat](https://onco.cc/drugs/belinostat/), [Autologous stem cell transplant (high-dose therapy)](https://onco.cc/technologies/autologous-stem-cell-transplant/), [Infection prophylaxis in lymphoma: PJP, herpes, fungal risk and vaccination](https://onco.cc/terms/lymphoma-tx-pjp-and-infection-prophylaxis/), [Stem cell transplant in lymphoma: what it is still for](https://onco.cc/terms/lymphoma-tx-transplant-role/))

## State of the art

- Angioimmunoblastic T-cell lymphoma grows out of clonal haematopoiesis. The TET2 and DNMT3A mutations it carries are present in non-tumour blood cells as well as in the lymphoma, which places them earlier, in the stem cell; the RHOA G17V substitution is found only in the tumour cells and is the later, lineage-defining event.
- RHOA G17V was reported in 68% of cases, and every case carrying it also carried a TET2 mutation. An independent series found it in 67% of angioimmunoblastic cases and 18% of peripheral T-cell lymphoma not otherwise specified, alongside IDH2, FYN, ATM, B2M and CD58 lesions.
- The mutated protein does not bind GTP and also blocks the normal copy, so it works against the wild-type protein rather than simply failing. The epigenetic lesions are the reason hypomethylating agents are used and studied here, although no test selects them.

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Angioimmunoblastic_T-cell_lymphoma
- NCI PDQ: adult non-Hodgkin lymphoma treatment: https://www.cancer.gov/types/lymphoma/patient/adult-nhl-treatment-pdq
- Alaggio 2022, Leukemia: the 5th edition WHO classification of haematolymphoid tumours, lymphoid neoplasms: https://doi.org/10.1038/s41375-022-01620-2
- Federico 2013, JCO: angioimmunoblastic T-cell lymphoma in the International Peripheral T-cell Lymphoma Project: https://doi.org/10.1200/jco.2011.37.3647
- Advani 2021, Blood: outcomes and prognostic factors in angioimmunoblastic T-cell lymphoma, the international T-cell Project: https://doi.org/10.1182/blood.2020010387

## Connected records

- cancers: [Cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome)](https://onco.cc/cancers/cutaneous-t-cell-lymphoma/), [Diffuse large B-cell lymphoma](https://onco.cc/cancers/dlbcl/), [Hepatosplenic T-cell lymphoma](https://onco.cc/cancers/hepatosplenic-t-cell-lymphoma/), [Peripheral T-cell lymphomas (including cutaneous T-cell lymphoma)](https://onco.cc/cancers/peripheral-t-cell-lymphoma/)
- biomarkers: [Immunoglobulin and T-cell receptor clonality](https://onco.cc/biomarkers/ig-tcr-clonality/), [RHOA G17V](https://onco.cc/biomarkers/rhoa-g17v/)
- technologies: [Autologous stem cell transplant (high-dose therapy)](https://onco.cc/technologies/autologous-stem-cell-transplant/), [Comprehensive genomic profiling](https://onco.cc/technologies/cgp/), [Early integrated palliative care](https://onco.cc/technologies/palliative-care/), [Exercise during chemotherapy and radiotherapy](https://onco.cc/technologies/exercise-during-chemotherapy/), [Histopathology & immunohistochemistry](https://onco.cc/technologies/histopathology-ihc/), [Immunoglobulin and T-cell receptor clonality testing](https://onco.cc/technologies/clonality-testing/), [Multidisciplinary tumour boards](https://onco.cc/technologies/multidisciplinary-tumour-board/), [Oncofertility and fertility preservation](https://onco.cc/technologies/fertility-preservation/), [Peer support and support groups](https://onco.cc/technologies/peer-support-groups/), [Prehabilitation before cancer surgery](https://onco.cc/technologies/prehabilitation/), [Psycho-oncology and distress screening](https://onco.cc/technologies/psycho-oncology/), [Survivorship care and late-effects surveillance](https://onco.cc/technologies/survivorship-care-plan/)
- targets: [B2M](https://onco.cc/targets/b2m/), [CD30](https://onco.cc/targets/cd30/), [CD52](https://onco.cc/targets/cd52/), [CD58](https://onco.cc/targets/cd58/), [DNA methyltransferase 3A (DNMT3A)](https://onco.cc/targets/dnmt3a/), [FYN](https://onco.cc/targets/fyn/), [RHOA](https://onco.cc/targets/rhoa/), [TET2](https://onco.cc/targets/tet2/)
- drugs: [Azacitidine](https://onco.cc/drugs/azacitidine/), [Belinostat](https://onco.cc/drugs/belinostat/), [Brentuximab vedotin](https://onco.cc/drugs/brentuximab-vedotin/), [Cyclophosphamide](https://onco.cc/drugs/cyclophosphamide/), [Doxorubicin](https://onco.cc/drugs/doxorubicin/), [Etoposide](https://onco.cc/drugs/etoposide/), [Pralatrexate](https://onco.cc/drugs/pralatrexate/), [Prednisone](https://onco.cc/drugs/prednisone/), [Romidepsin](https://onco.cc/drugs/romidepsin/), [Vincristine](https://onco.cc/drugs/vincristine/)
- pathways: [Clonal haematopoiesis (CHIP)](https://onco.cc/pathways/clonal-haematopoiesis/), [Epigenetic reprogramming](https://onco.cc/pathways/epigenetic-reprogramming/), [JAK-STAT signalling](https://onco.cc/pathways/jak-stat/)
- terms: [Cancer-related fatigue (tiredness)](https://onco.cc/terms/cancer-related-fatigue/), [Central venous access (port, PICC line)](https://onco.cc/terms/central-venous-access/), [Febrile neutropenia](https://onco.cc/terms/febrile-neutropenia/), [Financial toxicity](https://onco.cc/terms/financial-toxicity/), [Infection prophylaxis in lymphoma: PJP, herpes, fungal risk and vaccination](https://onco.cc/terms/lymphoma-tx-pjp-and-infection-prophylaxis/), [Late effects and survivorship toxicity](https://onco.cc/terms/late-effects/), [Neutropenia](https://onco.cc/terms/neutropenia/), [Prognostic Index for T-cell lymphoma (PIT)](https://onco.cc/terms/lymphoma-pit-score/), [R-CHOP (lymphoma chemoimmunotherapy)](https://onco.cc/terms/r-chop/), [Stem cell transplant in lymphoma: what it is still for](https://onco.cc/terms/lymphoma-tx-transplant-role/), [The lymphoma regimen alphabet: R-CHOP, pola-R-CHP, DA-EPOCH-R, ABVD, BEACOPP and the rest](https://onco.cc/terms/lymphoma-tx-regimen-alphabet/), [What it costs to aim at a lineage antigen](https://onco.cc/terms/lymphoma-bio-lineage-antigen-cost/)
- trials: [A Study of Duvelisib Versus Gemcitabine or Bendamustine in Participants With Relapsed/Refractory Nodal T Cell Lymphoma With T Follicular Helper (TFH) Phenotype](https://onco.cc/trials/nct06522737/)
- ideas: [Randomised evidence for the T-cell lymphomas, including the ones that are not in Europe or North America](https://onco.cc/ideas/lymphoma-ev-randomised-evidence-for-the-t-cell-lymphomas/)

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JSON: https://onco.cc/api/v1/entities/angioimmunoblastic-t-cell-lymphoma.json