Nodal T-follicular helper cell lymphoma, angioimmunoblastic type (angioimmunoblastic T-cell lymphoma)
Prepared with OnCo (onco.cc/prep/angioimmunoblastic-t-cell-lymphoma/). Orientation, not medical advice; your team knows your case.
My details
What I know, what is unclear, changes to discuss
Saved in this browserMy questions
13 on the sheet- 1.What is my exact diagnosis, stage, and grade, and which tests established them?
- 2.Which biomarkers have been tested on my tumour (for example 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), and what were the results?
- 3.Which subtype is my cancer, and does that change the recommended treatment?
- 4.Is germline (inherited) genetic testing recommended for me or my family?
- 5.For my situation (first line), which of the standard options do you recommend and why?
- 6.Am I a candidate for Cyclophosphamide, and what side effects should I expect?
- 7.For my situation (relapsed), which of the standard options do you recommend and why?
- 8.Am I a candidate for Romidepsin, Belinostat, Pralatrexate or related drugs, and what side effects should I expect?
- 9.How do the results of A Study of Duvelisib Versus Gemcitabine or Bendamustine in Participants With Relapsed/Refractory Nodal T Cell Lymphoma With T Follicular Helper (TFH) Phenotype apply to someone like me?
- 10.For my situation (angioimmunoblastic t-cell lymphoma: treated as a nodal peripheral t-cell lymphoma, with two differences), which of the standard options do you recommend and why?
- 11.Am I a candidate for Brentuximab vedotin, Cyclophosphamide, Doxorubicin or related drugs, and what side effects should I expect?
- 12.Would a second opinion at a high-volume centre change anything, and can you help arrange it?
- 13.What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?
The words I may hear
- What it costs to aim at a lineage antigen: Almost every lymphoma drug that finds the cancer by a surface marker finds healthy cells carrying the same marker.
- Prognostic Index for T-cell lymphoma (PIT): A four-item score that estimates the outlook in nodal T-cell lymphoma, built because the index used for B-cell lymphoma separated these patients poorly.
- Infection prophylaxis in lymphoma: PJP, herpes, fungal risk and vaccination: Several lymphoma treatments knock out the part of the immune system that keeps a particular lung infection, Pneumocystis, at bay.
- R-CHOP (lymphoma chemoimmunotherapy): R-CHOP is the standard first treatment for diffuse large B-cell lymphoma: rituximab (an antibody against CD20) plus four chemotherapy drugs (cyclophosphamide, doxorubicin, vincristine, prednisone), given every three weeks for six cycles with curative intent.
- Stem cell transplant in lymphoma: what it is still for: An autologous transplant is very high-dose chemotherapy followed by the patient's own stored stem cells to rescue the bone marrow.
- Central venous access (port, PICC line): A long-term line into a large vein near the heart, either a small disc under the skin (port) or a tube from the arm (PICC), so chemotherapy can be given and blood drawn without repeated needle sticks.
- Financial toxicity: The harm caused to patients by the cost of cancer care: depleted savings, debt, skipped medication and worse survival.
- The lymphoma regimen alphabet: R-CHOP, pola-R-CHP, DA-EPOCH-R, ABVD, BEACOPP and the rest: Lymphoma treatment is written in acronyms, one letter per drug.
- Neutropenia: A shortage of neutrophils, the white blood cells that fight bacteria, caused by chemotherapy hitting the bone marrow.
- Febrile neutropenia: Fever in a patient whose infection-fighting white cells have been wiped out by chemotherapy.
Tests and results to bring
Biomarker results to ask for: 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).
Scans and tests linked to this cancer: Comprehensive genomic profiling, Histopathology & immunohistochemistry, Multidisciplinary tumour boards.
Bring copies of scan reports, pathology and blood results, and a list of every medicine and supplement.
The treatments I may be offered
- 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), Cyclophosphamide, R-CHOP (lymphoma chemoimmunotherapy))
- 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, Brentuximab vedotin, Cyclophosphamide, Doxorubicin, Vincristine, Prednisone, Etoposide, Romidepsin, Belinostat, Autologous stem cell transplant (high-dose therapy), Infection prophylaxis in lymphoma: PJP, herpes, fungal risk and vaccination, Stem cell transplant in lymphoma: what it is still for)
- Relapsed: Romidepsin, belinostat or pralatrexate; azacitidine with romidepsin; duvelisib in the TFH-phenotype trial TERZO; brentuximab vedotin when CD30-positive. (Romidepsin, Belinostat, Pralatrexate, Azacitidine, 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)
From the standard of care recorded for this cancer; which apply depends on your stage and biomarkers. Ask which the team recommends and why.