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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.
Marginal zone lymphomas come in three forms. Extranodal (MALT) lymphoma arises in mucosal tissue chronically stimulated by infection or autoimmunity: gastric MALT lymphoma from Helicobacter pylori, ocular adnexal from Chlamydia in some regions, salivary gland in Sjogren's syndrome and thyroid in Hashimoto's disease; eradicating H. pylori puts most early gastric cases into remission, and localised disease elsewhere is cured with low-dose radiotherapy. Splenic marginal zone lymphoma presents with a large spleen and circulating villous lymphocytes and is linked to hepatitis C, whose treatment can induce remission; rituximab has replaced splenectomy. Nodal marginal zone lymphoma behaves like follicular lymphoma. Systemic treatment when needed is rituximab alone or with bendamustine or chlorambucil, lenalidomide-rituximab, and the BTK inhibitors ibrutinib and zanubrutinib for relapsed disease.
About one in ten non-Hodgkin lymphomas, the second commonest indolent type after follicular lymphoma; most patients live for many years and many are cured by treating the infection or inflammation that drove the disease.
Leukaemias, myeloma and MDS live in the marrow and blood; lymphomas grow in lymph nodes and spleen. The node stations are the disease map, not a route of spread, and staging counts them.
In lymphoma the node stations are the disease itself; staging (Ann Arbor / Lugano) counts how many regions and sides of the diaphragm are involved.
Same organ: High-grade B-cell lymphoma with MYC and BCL2 rearrangements (double-hit lymphoma), Mediastinal grey zone lymphoma, Primary effusion lymphoma, Plasmablastic lymphoma, T-cell/histiocyte-rich large B-cell lymphoma, EBV-positive diffuse large B-cell lymphoma, Primary large B-cell lymphoma of the testis, Gastric MALT lymphoma, Ocular adnexal MALT lymphoma, Extranodal NK/T-cell lymphoma, Adult T-cell leukaemia/lymphoma, ALK-positive anaplastic large cell lymphoma, ALK-negative anaplastic large cell lymphoma, Breast implant-associated anaplastic large cell lymphoma, Primary cutaneous anaplastic large cell lymphoma, Lymphomatoid papulosis, Mycosis fungoides, Enteropathy-associated T-cell lymphoma, Monomorphic epitheliotropic intestinal T-cell lymphoma, Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma), Splenic marginal zone lymphoma, Nodal marginal zone lymphoma, Primary cutaneous marginal zone lymphoma, Primary cutaneous follicle centre lymphoma, Sezary syndrome, Nodal T-follicular helper cell lymphoma, angioimmunoblastic type (angioimmunoblastic T-cell lymphoma), Hepatosplenic T-cell lymphoma, Intravascular large B-cell lymphoma, Lymphomatoid granulomatosis, T-cell prolymphocytic leukaemia, Splenic B-cell lymphoma/leukaemia with prominent nucleoli (formerly B-cell prolymphocytic leukaemia and hairy cell leukaemia variant), T-cell large granular lymphocytic leukaemia, Mixed-phenotype acute leukaemia, Myeloid leukaemia of Down syndrome, Burkitt leukaemia, Cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome), Primary mediastinal (thymic) large B-cell lymphoma, Leukaemia (all types), Acute myeloid leukaemia, Acute lymphoblastic leukaemia, Chronic lymphocytic leukaemia, Chronic myeloid leukaemia (CML), Diffuse large B-cell lymphoma, Follicular lymphoma, Hodgkin lymphoma, Mantle cell lymphoma, Multiple myeloma, Non-Hodgkin lymphoma (all types), Myelodysplastic syndromes / neoplasms (MDS), Myeloproliferative neoplasms (PV, ET, myelofibrosis), Polycythaemia vera (PV), Essential thrombocythaemia (ET), Waldenström macroglobulinaemia, Hairy cell leukaemia, Peripheral T-cell lymphomas (including cutaneous T-cell lymphoma), Blastic plasmacytoid dendritic cell neoplasm (BPDCN), Burkitt lymphoma, HIV-associated (AIDS-related) lymphomas, Chronic myelomonocytic leukaemia and MDS/MPN overlap neoplasms, Systemic mastocytosis, Erdheim-Chester disease, Rosai-Dorfman disease and other histiocytic neoplasms, Langerhans cell histiocytosis (LCH), Post-transplant lymphoproliferative disorder (PTLD), FLT3-mutated acute myeloid leukaemia, IDH1- and IDH2-mutated acute myeloid leukaemia, NPM1-mutated and KMT2A-rearranged acute myeloid leukaemia, Secondary and therapy-related acute myeloid leukaemia, Acute promyelocytic leukaemia, Acute myeloid leukaemia in older or unfit patients, Smouldering multiple myeloma, Newly diagnosed multiple myeloma, transplant-eligible, Newly diagnosed multiple myeloma, transplant-ineligible, Relapsed or refractory multiple myeloma, Plasma cell leukaemia, Lower-risk myelodysplastic syndromes, Higher-risk myelodysplastic syndromes, Chronic lymphocytic leukaemia, first treatment, Relapsed or refractory chronic lymphocytic leukaemia, Richter transformation of chronic lymphocytic leukaemia, Chronic myeloid leukaemia, chronic phase, Chronic myeloid leukaemia, accelerated and blast phase, Primary myelofibrosis, Standard-risk B-cell acute lymphoblastic leukaemia in children, High-risk acute lymphoblastic leukaemia in children (high-risk B-ALL and T-ALL), Philadelphia chromosome-positive acute lymphoblastic leukaemia in children (Ph-positive ALL), Philadelphia chromosome-like acute lymphoblastic leukaemia (Ph-like or BCR::ABL1-like ALL), Infant acute lymphoblastic leukaemia (KMT2A-rearranged, under one year), Relapsed and refractory acute lymphoblastic leukaemia in children, Acute myeloid leukaemia in children, Erdheim-Chester disease, Rosai-Dorfman-Destombes disease, Single-system Langerhans cell histiocytosis (bone, skin or one other organ), Multisystem Langerhans cell histiocytosis (with or without risk-organ involvement), Indolent and smouldering systemic mastocytosis, Advanced systemic mastocytosis (aggressive SM, SM with an associated haematological neoplasm, mast cell leukaemia), Early-stage classical Hodgkin lymphoma (stage I to II), Advanced-stage classical Hodgkin lymphoma (stage III to IV), Nodular lymphocyte-predominant Hodgkin lymphoma (nodular lymphocyte-predominant B-cell lymphoma), Relapsed and refractory classical Hodgkin lymphoma
Nothing recorded yet.
Nothing recorded yet.
Also on OnCo: Symptoms and red flags · Early detection roadmap.
Eradication therapy and endoscopic follow-up; radiotherapy if the lymphoma persists or carries t(11;18).
Low-dose involved-site radiotherapy (as little as 4 Gy in two fractions for some sites); surgery rarely.
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.
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.
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.
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A new combination for relapsed or refractory follicular lymphoma that adds a CD19-directed antibody to the established lenalidomide and rituximab pairing, with the largest progression-free survival hazard ratio reported in the setting.
Axicabtagene ciloleucel as an option in relapsed follicular and marginal zone lymphoma. Compared with tisagenlecleucel in ELARA, the response rates are higher and the neurological toxicity substantially greater, which is the trade-off a patient and centre weigh.
Every lymphoma diagnosis on this site refers to an entity in this classification or the parallel International Consensus Classification.
Confirms 24 Gy in 12 fractions as the optimal dose for indolent lymphoma when durable local control is the goal.
Zanubrutinib is an approved option for relapsed marginal zone lymphoma after anti-CD20 therapy, chosen for its tolerability profile.
The piece that turns a research classification into something a trial can use on one person's biopsy, with a probability attached rather than a flat label. It is the reason genetics-directed lymphoma trials became possible at all.
The site-specific and stage-specific approach on the marginal zone lymphoma page follows this guideline.
Lenalidomide-rituximab is approved for relapsed follicular and marginal zone lymphoma and is a standard chemotherapy-free choice, though marginal zone-specific evidence is thinner.
Query for this cancer: (TITLE:"Marginal zone lymphoma" OR ABSTRACT:"Marginal zone lymphoma" OR TITLE:"MZL" OR ABSTRACT:"MZL" OR TITLE:"MALT lymphoma" OR ABSTRACT:"MALT lymphoma" OR TITLE:"Extranodal marginal zone lymphoma" OR ABSTRACT:"Extranodal marginal zone lymphoma" OR TITLE:"Splenic marginal zone lymphoma" OR ABSTRACT:"Splenic marginal zone lymphoma" OR TITLE:"Nodal marginal zone lymphoma" OR ABSTRACT:"Nodal marginal zone lymphoma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Marginal zone lymphoma, not a curated reading list.
The targets of this cancer's medicines and the ones linked to it directly.
Cases by country, the UK and NHS pathway and other country lenses, the expert centres with trials on record, and the centres named on this cancer's subtypes.
One section per setting: the options named, what each is for, the trials behind them, the recorded trade-offs and the questions to ask.
Lymphoma Action says a small number of people have more serious problems such as seizures or swelling of the brain, treated with steroids and intensive care, and that most improve within a few days of treatment starting. This is 999.
Breathing very fast, confusion or slurred speech, blue, pale or blotchy skin, a very high or very low temperature, shivering, or a rash that does not fade when pressed: the NHS says call 999 or go to A and E, and do not drive yourself.
Bleeding that does not stop by itself, bleeding from more than one site, or new bruising in several places or one large area.
Blood in stool or urine, vomiting blood, a bleed that will not stop, or a severe headache; fatal bleeding events have occurred and the labels advise considering the risk around surgery and with blood thinners.
A swollen painful calf, or sudden breathlessness with chest pain; venous and arterial thromboembolism is a boxed warning and blood-thinning prophylaxis is recommended.
Fever of 38 C or higher is grade 1 CRS; fever with low blood pressure, fast heartbeat, breathlessness or low oxygen is grade 2 or higher. The labels carry a boxed warning and say to report fever immediately.
See all on the product pages:BendamustineCD20 bispecific antibodies: step-up dosing, fixed duration and what it is like to take oneCentral venous access (port, PICC line)ChlorambucilCytokine release syndrome (CRS)Cytokine release syndrome and ICANS: grading and managementFebrile neutropeniaHypogammaglobulinaemia and infection risk after B-cell therapiesIbrutinibICANS (neurotoxicity)LenalidomideLisocabtagene maraleucelNeutropeniaThe CAR-T pathway in lymphoma: referral, apheresis, bridging and the waitingZanubrutinib·Printable cards in the navigator
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