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MALT lymphoma is a slow-growing lymphoma that starts in lymphoid tissue lining an organ, most often the stomach, where it is usually caused by long-standing Helicobacter pylori infection and can be cured with antibiotics alone. Other sites include the eye socket, salivary glands, thyroid, lung and skin; localised disease is treated with low-dose radiotherapy and widespread disease with rituximab.
WHO-HAEM5 keeps extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue as the extranodal member of the marginal zone lymphoma family, arising in tissue chronically inflamed by infection or autoimmunity: Helicobacter pylori in the stomach, Sjogren syndrome in the salivary glands, Hashimoto thyroiditis in the thyroid, and Chlamydia psittaci in some ocular adnexal cases (Alaggio 2022; Zucca 2020). The ESMO guideline recommends H. pylori eradication as first treatment for gastric MALT lymphoma whatever the stage, with the t(11;18) translocation predicting failure of antibiotics; even H. pylori-negative gastric disease can respond, with 12 of 13 patients alive and several in remission after antibiotics alone in one centre's series with a median follow-up of 95 months (Annals of Hematology 2015). Whether FDG-PET is useful for staging remains unsettled, with conflicting avidity across 32 studies (Clin Lymphoma Myeloma Leuk 2020).
How it differs from its parent: the parent page covers the three marginal zone lymphomas together; the extranodal type is the one with an infectious cause to remove, the one treated with involved-site radiotherapy at 24 Gy or less when localised, and the one with organ-specific presentations (gastritis, a salivary mass, an orbital swelling, lung nodules).
How common: the ESMO guideline gives marginal zone lymphomas as about 5 to 15 percent of non-Hodgkin lymphomas depending on region, with the extranodal type the largest share; no UK figure for the type alone is in the sources read.
Treatment: H. pylori eradication for gastric disease; involved-site radiotherapy for localised disease at other sites; rituximab alone or with chlorambucil or bendamustine for disseminated or relapsed disease; BTK inhibitors (zanubrutinib) and lenalidomide-rituximab as later lines, as on the parent page (Zucca 2020).
The commonest marginal zone lymphoma; marginal zone lymphomas together are the third most common non-Hodgkin lymphoma group in the ESMO guideline, and the stomach is the most common MALT site (Zucca 2020; Clin Lymphoma Myeloma Leuk 2020). Cancer Research UK lists marginal zone lymphomas among the low-grade types.
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, 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, Marginal zone lymphoma, 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
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Also on OnCo: Symptoms and red flags · Early detection roadmap.
H. pylori eradication first, with endoscopic follow-up; radiotherapy if the lymphoma persists.
Involved-site radiotherapy (the FoRT trial tested the dose in follicular and marginal zone lymphoma).
Rituximab alone or with chlorambucil or bendamustine; zanubrutinib or lenalidomide-rituximab later (AUGMENT; MAHOGANY).
Helicobacter pylori eradication is the first treatment for every Helicobacter-positive gastric MALT lymphoma, whatever the stage, and it is a cure in most of them. The observation that made this possible was published in 1993, when eradication produced regression of the lymphoma in five of six patients, and it remains one of the very few instances in oncology in which a course of antibiotics is the definitive treatment of a malignancy. What is given: a standard triple or quadruple eradication regimen chosen by local resistance patterns, typically a proton pump inhibitor with two antibiotics for 10 to 14 days. Eradication is then confirmed, by urea breath test or stool antigen at least four weeks after antibiotics and two weeks off the proton pump inhibitor, because failure of eradication is the commonest reason for failure of the lymphoma to respond. What happens next: endoscopic and histological follow-up every three to six months. Regression is slow and can take twelve to eighteen months, so persistent histological disease in a patient whose Helicobacter has been eradicated and who has no symptoms is watched, not treated. The t(11;18) translocation predicts failure to respond to eradication and is worth testing where it is available. Helicobacter-negative gastric MALT lymphoma is still given eradication therapy in many centres, because some respond, but it is not relied on.
Where the Helicobacter has been eradicated and the lymphoma persists and is causing symptoms or progressing, radiotherapy is the next treatment: about 24 Gy in 12 fractions to the stomach, which controls the disease in the great majority. Systemic treatment, usually rituximab alone or rituximab with chlorambucil or bendamustine, is used for disseminated disease or where radiotherapy is not possible. Surgery has no routine role. MALT lymphoma at other sites, salivary gland, thyroid, lung, skin, orbit, breast, is treated on the same principle: a localised, symptomatic site gets 24 Gy, an asymptomatic one can be watched, and disseminated disease gets rituximab-based systemic treatment. Ocular adnexal MALT is sometimes given doxycycline first for Chlamydia psittaci, with response rates that vary widely by region. Salivary gland MALT in Sjogren syndrome and thyroid MALT in Hashimoto thyroiditis are managed jointly with the specialty that treats the underlying autoimmune disease. The IELSG-19 trial is the randomised evidence for systemic treatment: five-year event-free survival was 68 per cent with chlorambucil and rituximab, 51 per cent with chlorambucil alone and 50 per cent with rituximab alone, with five-year overall survival of about 90 per cent in each arm.
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Query for this cancer: (TITLE:"Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue" OR ABSTRACT:"Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue" OR TITLE:"MALT lymphoma" OR ABSTRACT:"MALT lymphoma" OR TITLE:"Mucosa-associated lymphoid tissue lymphoma" OR ABSTRACT:"Mucosa-associated lymphoid tissue lymphoma" OR TITLE:"Extranodal marginal zone lymphoma" OR ABSTRACT:"Extranodal marginal zone lymphoma" OR TITLE:"Extranodal marginal zone lymphoma of MALT" OR ABSTRACT:"Extranodal marginal zone lymphoma of MALT" OR TITLE:"Gastric MALT lymphoma" OR ABSTRACT:"Gastric MALT lymphoma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma), not a curated reading list.
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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.
A swollen painful calf, or sudden breathlessness with chest pain; venous and arterial thromboembolism is a boxed warning and blood-thinning prophylaxis is recommended.
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.
Macmillan lists difficulty passing urine, loss of bladder or bowel control and constipation among the signs of spinal cord compression and says to contact the hospital straight away. If you cannot reach anyone, go to A and E and say you have lymphoma and symptoms of spinal cord compression.
Yellowing during or after antibody treatment for a B-cell lymphoma is a reason to be seen the same day and to have liver blood tests, not to wait for the next cycle.
See all on the product pages:BendamustineCentral venous access (port, PICC line)ChlorambucilFebrile neutropeniaHepatitis B reactivation before rituximab and other anti-CD20 antibodiesHypogammaglobulinaemia and infection risk after B-cell therapiesLenalidomideMetastatic spinal cord compression (MSCC)NeutropeniaZanubrutinib·Printable cards in the navigator
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