Nodal marginal zone lymphoma
Prepared with OnCo (onco.cc/prep/nodal-marginal-zone-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
11 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 Exclusion immunophenotype: CD20 positive, CD5, CD10, cyclin D1 and BCL6 negative, KMT2D, PTPRD, NOTCH2 and KLF2 mutations, Absence of extranodal or splenic disease on staging), 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 (all stages), which of the standard options do you recommend and why?
- 6.Am I a candidate for Rituximab, Bendamustine, Lenalidomide or related drugs, and what side effects should I expect?
- 7.For my situation (nodal marginal zone lymphoma: treated like follicular lymphoma, with the same choices), which of the standard options do you recommend and why?
- 8.Am I a candidate for Rituximab, Bendamustine, Lenalidomide or related drugs, and what side effects should I expect?
- 9.How do the results of AUGMENT apply to someone like me?
- 10.Would a second opinion at a high-volume centre change anything, and can you help arrange it?
- 11.What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?
The words I may hear
- Living with an indolent lymphoma rather than being cured of one: Slow-growing lymphomas are usually controlled rather than cured: treatment works, the disease goes away for a while, and at some point it comes back and is treated again.
- Watch and wait in follicular lymphoma: being told you have cancer and that nobody will treat it: For a slow-growing lymphoma that is not causing problems, treating straight away has not been shown to help people live longer, so the usual plan is regular checks and treatment later.
- Watchful waiting, and how it differs from active surveillance: Two things that sound the same and are not.
- Low antibodies and infection risk for years after anti-CD20 and bispecific antibodies: Treatments that remove B cells also remove the cells that make antibodies, and the effect can last for years after the last dose.
- Watch and wait in lymphoma: when the right treatment is none yet: For slow-growing lymphomas that are not causing symptoms, treating straight away does not help people live longer.
- Hypogammaglobulinaemia and infection risk after B-cell therapies: A shortage of antibodies because treatment has wiped out the B cells or plasma cells that make them, as CD19 CAR-T, CD20 antibodies and BCMA therapies deliberately do.
- Radiotherapy in lymphoma: involved-site fields, 24 Gy, 4 Gy and total skin electron therapy: Lymphoma is one of the most radiation-sensitive cancers there is, so the doses are low and the fields are small.
- 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.
- Neutropenia: A shortage of neutrophils, the white blood cells that fight bacteria, caused by chemotherapy hitting the bone marrow.
Tests and results to bring
Biomarker results to ask for: Exclusion immunophenotype: CD20 positive, CD5, CD10, cyclin D1 and BCL6 negative, KMT2D, PTPRD, NOTCH2 and KLF2 mutations, Absence of extranodal or splenic disease on staging.
Scans and tests linked to this cancer: 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
- All stages: Treated as follicular lymphoma of the same stage: radiotherapy when localised, rituximab with or without chemotherapy when advanced, lenalidomide-rituximab or BTK inhibitors on relapse. (Follicular lymphoma, Rituximab, Bendamustine, Lenalidomide, Zanubrutinib)
- Nodal marginal zone lymphoma: treated like follicular lymphoma, with the same choices: Nodal marginal zone lymphoma has no site-specific treatment of its own and is managed on the follicular lymphoma pathway: watch and wait while asymptomatic, involved-site radiotherapy at 24 Gy for genuinely localised disease, and rituximab-based chemoimmunotherapy (bendamustine and rituximab, or R-CVP) when the disease causes symptoms, organ compromise or cytopenias. Lenalidomide with rituximab is supported by AUGMENT, which enrolled marginal zone as well as follicular lymphoma and gave median progression-free survival of 39.4 against 14.1 months for rituximab alone. Zanubrutinib is an option at relapse on the strength of MAGNOLIA. Hepatitis C should be tested for, as in the splenic form. Transformation to diffuse large B-cell lymphoma is treated as aggressive lymphoma. (Rituximab, Bendamustine, Lenalidomide, Zanubrutinib, AUGMENT, Watch and wait in lymphoma: when the right treatment is none yet, Radiotherapy in lymphoma: involved-site fields, 24 Gy, 4 Gy and total skin electron therapy, MAGNOLIA: zanubrutinib in relapsed or refractory marginal zone lymphoma)
From the standard of care recorded for this cancer; which apply depends on your stage and biomarkers. Ask which the team recommends and why.