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6 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
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.
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.
| Setting | Approach | Guideline |
|---|---|---|
| First line | CHOP-based chemotherapy, with etoposide in younger patients, and autologous transplant in first complete remission for fit patients (T-cell Project data). | not mapped |
| Relapsed | Romidepsin, belinostat or pralatrexate; azacitidine with romidepsin; duvelisib in the TFH-phenotype trial TERZO; brentuximab vedotin when CD30-positive. | not mapped |
| 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. | not mapped |