Splenic marginal zone lymphoma
Prepared with OnCo (onco.cc/prep/splenic-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
12 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 Villous lymphocytes and marrow intrasinusoidal infiltration, NOTCH2, KLF2 and TP53 mutations, Hepatitis C serology, Immunophenotype: CD20 positive, CD5, CD10, CD103 and annexin A1 negative), 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 (asymptomatic), which of the standard options do you recommend and why?
- 6.For my situation (symptomatic), which of the standard options do you recommend and why?
- 7.Am I a candidate for Rituximab, Bendamustine, Zanubrutinib or related drugs, and what side effects should I expect?
- 8.How do the results of AUGMENT apply to someone like me?
- 9.For my situation (splenic marginal zone lymphoma: hepatitis c first, then rituximab, then splenectomy), which of the standard options do you recommend and why?
- 10.Am I a candidate for Rituximab, Bendamustine, Zanubrutinib, and what side effects should I expect?
- 11.Would a second opinion at a high-volume centre change anything, and can you help arrange it?
- 12.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.
- 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.
- 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.
- 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: Villous lymphocytes and marrow intrasinusoidal infiltration, NOTCH2, KLF2 and TP53 mutations, Hepatitis C serology, Immunophenotype: CD20 positive, CD5, CD10, CD103 and annexin A1 negative.
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
- Asymptomatic: Watch and wait; antiviral therapy first if hepatitis C is present. (Marginal zone lymphoma)
- Symptomatic: Rituximab alone or with chemotherapy; splenectomy for selected patients; BTK inhibitors or lenalidomide-rituximab later. (Rituximab, Bendamustine, Zanubrutinib, Lenalidomide, AUGMENT)
- Splenic marginal zone lymphoma: hepatitis C first, then rituximab, then splenectomy: An indolent disease of the spleen, marrow and blood. A patient with no symptoms, no cytopenias and a spleen that is not troublesome is watched, sometimes for years. When treatment is needed, the order is: test and treat hepatitis C if present, because direct-acting antivirals alone produce lymphoma remission in a proportion of hepatitis C-associated cases and that is a reason to check before giving any chemotherapy. Then rituximab alone, weekly for four to eight doses, which corrects cytopenias and shrinks the spleen in the large majority and has displaced splenectomy as first treatment. Chemoimmunotherapy with bendamustine and rituximab is used for disease that does not respond or that transforms. Splenectomy is now reserved for patients who cannot have rituximab, whose disease is confined to the spleen and who need a diagnosis, or who have refractory painful splenomegaly. It requires pneumococcal, Haemophilus influenzae type b and meningococcal vaccination at least two weeks beforehand where possible, and lifelong penicillin prophylaxis afterwards. Zanubrutinib has activity in relapsed disease across marginal zone subtypes. (Rituximab, Bendamustine, Zanubrutinib, Watch and wait in lymphoma: when the right treatment is none yet, Infection prophylaxis in lymphoma: PJP, herpes, fungal risk and vaccination, MAGNOLIA: zanubrutinib in relapsed or refractory marginal zone lymphoma, Marginal zone lymphomas: ESMO clinical practice guidelines)
From the standard of care recorded for this cancer; which apply depends on your stage and biomarkers. Ask which the team recommends and why.