Marginal zone lymphoma
Prepared with OnCo (onco.cc/prep/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
20 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 Helicobacter pylori status, tMALT1 translocation, Hepatitis C serology, MYD88 wild-type, Plasmacytic differentiation and paraprotein), 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 (gastric malt lymphoma, h. pylori-positive), which of the standard options do you recommend and why?
- 6.For my situation (localised extranodal disease), which of the standard options do you recommend and why?
- 7.For my situation (splenic marginal zone lymphoma), which of the standard options do you recommend and why?
- 8.Am I a candidate for Rituximab, and what side effects should I expect?
- 9.For my situation (advanced or relapsed), which of the standard options do you recommend and why?
- 10.Am I a candidate for Rituximab, Bendamustine, Chlorambucil or related drugs, and what side effects should I expect?
- 11.For my situation (marginal zone lymphoma: three different diseases under one name), which of the standard options do you recommend and why?
- 12.Am I a candidate for Rituximab, and what side effects should I expect?
- 13.For my situation (marginal zone lymphoma that is advanced, symptomatic or has relapsed), which of the standard options do you recommend and why?
- 14.Am I a candidate for Rituximab, Bendamustine, Chlorambucil or related drugs, and what side effects should I expect?
- 15.How do the results of AUGMENT apply to someone like me?
- 16.Are there clinical trials I could join, for example of Zanubrutinib, Lenalidomide?
- 17.Would a second opinion at a high-volume centre change anything, and can you help arrange it?
- 18.What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?
- 19.I read that “Transformation to diffuse large B-cell lymphoma in a minority”. How does that affect my plan?
- 20.I read that “No standard sequence of therapies is proven by randomised trials”. How does that affect my plan?
The words I may hear
- SS18::SSX fusion (synovial sarcoma): Every true synovial sarcoma carries a fusion between the SS18 gene on chromosome 18 and an SSX gene on the X chromosome; finding it by FISH, RNA sequencing or a newer antibody stain confirms a diagnosis that pathologists otherwise struggle with, and it also flags the tumour for the MAGE-A4 and NY-ESO-1 cell therapies that work almost only in this disease.
- POD24: progression of follicular lymphoma within two years, and why it changes the plan: Most follicular lymphoma comes back slowly and is treated again without much loss of life expectancy.
- CD20 bispecific antibodies: step-up dosing, fixed duration and what it is like to take one: Bispecific antibodies grab a lymphoma cell with one arm and a T cell with the other.
- Helicobacter pylori eradication as cancer treatment in gastric MALT lymphoma: Gastric MALT lymphoma is grown by a stomach bacterium, and killing the bacterium with a ten to fourteen day course of antibiotics cures most cases.
- LymphGen and the genetic clusters of large B-cell lymphoma: Sequencing shows that large B-cell lymphoma is at least seven diseases, each defined by which faults occur together.
- Transformation: when a slow lymphoma turns into a fast one: An indolent lymphoma can change into an aggressive one, usually by acquiring new genetic faults in the same clone.
- Hepatitis B reactivation before rituximab and other anti-CD20 antibodies: Anyone who has ever had hepatitis B can have the virus wake up when rituximab strips out their B cells, sometimes months later and sometimes fatally.
- Staging a lymphoma of the stomach or bowel: A lymphoma that starts in the stomach or bowel is staged by how deep it goes into the wall and how far along the lymph node chain it has travelled, not only by how many node regions are involved.
- M-protein, immunofixation and serum free light chains: Myeloma cells are clones of one antibody-making cell, so they pour a single identical antibody, the M-protein, into the blood; measuring it (and the free light chains that go with it) is how myeloma is diagnosed, how deep a response is judged, and how relapse is caught before symptoms.
- Transformation of an indolent lymphoma: A slow-growing lymphoma can change into a fast-growing one.
Tests and results to bring
Biomarker results to ask for: Helicobacter pylori status (gastric MALT), t(11;18) MALT1 translocation (predicts failure of eradication therapy), Hepatitis C serology (splenic), MYD88 wild-type (distinguishes from Waldenstrom), Plasmacytic differentiation and paraprotein.
Scans and tests linked to this cancer: Cytogenetics and FISH, Multidisciplinary tumour boards, Multiparameter flow cytometry MRD.
Bring copies of scan reports, pathology and blood results, and a list of every medicine and supplement.
The treatments I may be offered
- Localised extranodal disease: Low-dose involved-site radiotherapy (as little as 4 Gy in two fractions for some sites); surgery rarely. (IMRT / IGRT (modern external beam), Hypofractionated radiotherapy)
- Gastric MALT lymphoma, H. pylori-positive: Eradication therapy and endoscopic follow-up; radiotherapy if the lymphoma persists or carries t(11;18). (Non-Hodgkin lymphoma (all types))
- Splenic marginal zone lymphoma: Watch and wait if asymptomatic; antiviral therapy if hepatitis C-positive; rituximab alone or with chemotherapy; splenectomy now rare. (Rituximab)
- Marginal zone lymphoma: three different diseases under one name: Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT) arises at a site of chronic inflammation, most often the stomach, and is the one that can sometimes be cured with antibiotics. Splenic marginal zone lymphoma presents with a large spleen, cytopenias and circulating villous lymphocytes, and is associated with hepatitis C. Nodal marginal zone lymphoma behaves much like follicular lymphoma and is treated like it. The three share a cell of origin and very little else in the way of treatment, so the first question is which one it is. Two tests change the plan at diagnosis. Helicobacter pylori status in gastric MALT, because eradication is the first treatment. Hepatitis C status in splenic and nodal disease, because antiviral treatment alone can produce lymphoma remission in hepatitis C-associated cases. Other site-specific associations are recorded and occasionally actionable: Chlamydia psittaci in ocular adnexal MALT, Borrelia burgdorferi in cutaneous MALT, Campylobacter jejuni in immunoproliferative small intestinal disease. (Marginal zone lymphomas: ESMO clinical practice guidelines, Helicobacter pylori eradication as cancer treatment in gastric MALT lymphoma, Watch and wait in lymphoma: when the right treatment is none yet, Rituximab)
- Advanced or relapsed: Rituximab with bendamustine or chlorambucil, lenalidomide-rituximab; zanubrutinib or ibrutinib for relapsed disease. (Rituximab, Bendamustine, Chlorambucil, Lenalidomide, Zanubrutinib, Ibrutinib)
- Marginal zone lymphoma that is advanced, symptomatic or has relapsed: Treatment is indicated for symptoms, organ compromise, cytopenias or rapid progression, not for the presence of disease. Rituximab alone produces responses in about half. Chemoimmunotherapy with bendamustine and rituximab is the usual choice when more is needed, and rituximab with chlorambucil has the only randomised evidence in MALT (IELSG-19: five-year event-free survival 68 per cent for the combination against 51 per cent for chlorambucil and 50 per cent for rituximab alone, with five-year overall survival about 90 per cent in each arm, so the combination delays events without changing survival). At relapse, the BTK inhibitors are the newest class: MAGNOLIA treated relapsed or refractory marginal zone lymphoma of all subtypes with zanubrutinib and reported an objective response of 68 per cent, complete response 26 per cent and 15-month progression-free survival of 83 per cent, with fewer cardiac events than ibrutinib. Lenalidomide with rituximab is an option and marginal zone patients were included in AUGMENT. Lisocabtagene maraleucel received United States approval for relapsed or refractory marginal zone lymphoma after two or more prior lines on 4 December 2025. Local radiotherapy at 24 Gy remains the right answer for a single symptomatic site whatever the line. (Rituximab, Bendamustine, Chlorambucil, Zanubrutinib, Ibrutinib, Lenalidomide, Lisocabtagene maraleucel, AUGMENT, Radiotherapy in lymphoma: involved-site fields, 24 Gy, 4 Gy and total skin electron therapy, 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.