The first 60 days: Marginal zone lymphoma
Marginal zone lymphoma is a slow B-cell lymphoma that often grows where the body has been fighting a chronic infection: the stomach with Helicobacter pylori, the eye, the skin or the spleen. Curing the infection cures many early cases; the rest are treated with rituximab, chemotherapy or BTK inhibitors. Below, week by week, is what OnCo's record of Marginal zone lymphoma says about the first two months: the order is typical, the timing is yours to ask about. Sections appear only where the record has something to say. Orientation, not medical advice.
What happens now
Staging tests establish exactly what and where the cancer is. Everything else follows from the answers.
Ask which of these were tested and what the results were; see the report reader for what each value means.
These specialties appear in the standard of care for this cancer. In most centres they meet weekly as a tumour board to agree each plan; you can ask when yours was discussed and what was decided.
- SurgeonNamed in the standard of care for: Localised extranodal disease, Splenic marginal zone lymphoma.
- Medical oncologistNamed in the standard of care for: Gastric MALT lymphoma, H. pylori-positive, Localised extranodal disease, Splenic marginal zone lymphoma, Advanced or relapsed.
- Clinical oncologist (radiotherapy)Named in the standard of care for: Gastric MALT lymphoma, H. pylori-positive, Localised extranodal disease.
A second opinion from a centre that treats many similar cases is normal, not rude; the standard of care tells you what to compare it against.
Each row is a setting from the standard of care, in the order it usually arises. Not all will apply to you; your stage and biomarkers decide which do. The guideline grade, where recorded, says how strong the evidence is.
Low-dose involved-site radiotherapy (as little as 4 Gy in two fractions for some sites); surgery rarely.
Eradication therapy and endoscopic follow-up; radiotherapy if the lymphoma persists or carries t(11;18).
Watch and wait if asymptomatic; antiviral therapy if hepatitis C-positive; rituximab alone or with chemotherapy; splenectomy now rare.
Rituximab with bendamustine or chlorambucil, lenalidomide-rituximab; zanubrutinib or ibrutinib for relapsed disease.
Generated from this cancer's standard of care, biomarkers and open problems. Take the group that matches where you are. To tick, add your own and print, open the one-page appointment sheet.
Newly diagnosed
- What is my exact diagnosis, stage, and grade, and which tests established them?Everything else follows from an accurate stage and subtype.
- 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?These results decide eligibility for targeted therapy, immunotherapy, and trials.
- Which subtype is my cancer, and does that change the recommended treatment?Recognised subtypes for this cancer include Extranodal marginal zone lymphoma of MALT, Splenic marginal zone lymphoma, Nodal marginal zone lymphoma.
- Is germline (inherited) genetic testing recommended for me or my family?Inherited variants can change treatment and matter for relatives.
Gastric MALT lymphoma, H. pylori-positive
- For my situation (gastric malt lymphoma, h. pylori-positive), which of the standard options do you recommend and why?Guideline options include: Eradication therapy and endoscopic follow-up; radiotherapy if the lymphoma persists or carries t(11;18).
Localised extranodal disease
- For my situation (localised extranodal disease), which of the standard options do you recommend and why?Guideline options include: Low-dose involved-site radiotherapy (as little as 4 Gy in two fractions for some sites); surgery rarely.
Splenic marginal zone lymphoma
- For my situation (splenic marginal zone lymphoma), which of the standard options do you recommend and why?Guideline options include: Watch and wait if asymptomatic; antiviral therapy if hepatitis C-positive; rituximab alone or with chemotherapy; splenectomy now rare.
- Am I a candidate for Rituximab, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Advanced or relapsed
- For my situation (advanced or relapsed), which of the standard options do you recommend and why?Guideline options include: Rituximab with bendamustine or chlorambucil, lenalidomide-rituximab; zanubrutinib or ibrutinib for relapsed disease.
- Am I a candidate for Rituximab, Bendamustine, Chlorambucil or related drugs, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Any stage
- Are there clinical trials I could join, for example of Zanubrutinib, Lenalidomide?Trials are how the next standard of care is set; asking early keeps options open.
- Would a second opinion at a high-volume centre change anything, and can you help arrange it?Rare or high-stakes decisions benefit from a centre that treats many similar patients.
- What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?Supportive care improves quality of life and helps patients complete treatment.
- I read that “Transformation to diffuse large B-cell lymphoma in a minority”. How does that affect my plan?Open problems are where trials and second opinions matter most.
- I read that “No standard sequence of therapies is proven by randomised trials”. How does that affect my plan?Open problems are where trials and second opinions matter most.
Much of what helps in the first weeks is free if you know to ask: testing, helplines, rides and lodging, second opinions, trial travel.
- Marginal zone lymphoma: the full pageMarginal zone lymphoma is a slow B-cell lymphoma that often grows where the body has been fighting a chronic infection: the stomach with Helicobacter pylori, the eye, the skin or the spleen. Curing the infection cures many early cases; the rest are treated with rituximab, chemotherapy or BTK inhibitors.
- One-page appointment sheetYour questions, the words you may hear, what to bring, and space for the answers. Print it.
- Treatment sequencingWhich treatment tends to follow which, line by line.
- NavigatorStandard of care for your stage, what you have tried, and trials near you.