{"entity":{"id":"marginal-zone-lymphoma","kind":"cancer","name":"Marginal zone lymphoma","aka":["MZL","MALT lymphoma","Extranodal marginal zone lymphoma","Splenic marginal zone lymphoma","Nodal marginal zone lymphoma"],"tldr":"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.","summary":"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.","asOf":"2026-09-17","wikipedia":"https://en.wikipedia.org/wiki/Marginal_zone_B-cell_lymphoma","links":[{"label":"Wikipedia","url":"https://en.wikipedia.org/wiki/Marginal_zone_B-cell_lymphoma"}],"tags":["subtype-page"],"related":[],"cancers":[],"sections":[],"technologies":[],"targets":[],"drugs":[],"companies":[],"institutions":[],"pathways":[],"terms":[],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"notes":[],"group":"haematologic","burden":"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.","subtypes":["Extranodal marginal zone lymphoma of MALT (gastric, ocular adnexal, salivary, thyroid, lung, skin)","Splenic marginal zone lymphoma","Nodal marginal zone lymphoma","Paediatric nodal marginal zone lymphoma (indolent)"],"biomarkers":["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"],"standardOfCare":[{"setting":"Gastric MALT lymphoma, H. pylori-positive","approach":"Eradication therapy and endoscopic follow-up; radiotherapy if the lymphoma persists or carries t(11;18).","refs":["non-hodgkin-lymphoma"]},{"setting":"Localised extranodal disease","approach":"Low-dose involved-site radiotherapy (as little as 4 Gy in two fractions for some sites); surgery rarely.","refs":["imrt-igrt","hypofractionated-radiotherapy"]},{"setting":"Splenic marginal zone lymphoma","approach":"Watch and wait if asymptomatic; antiviral therapy if hepatitis C-positive; rituximab alone or with chemotherapy; splenectomy now rare.","refs":["rituximab"]},{"setting":"Advanced or relapsed","approach":"Rituximab with bendamustine or chlorambucil, lenalidomide-rituximab; zanubrutinib or ibrutinib for relapsed disease.","refs":["rituximab","bendamustine","chlorambucil","lenalidomide","zanubrutinib","ibrutinib"]}],"stateOfArt":["Gastric MALT lymphoma is the clearest example of a cancer cured by treating an infection.","Very low-dose radiotherapy controls localised disease with almost no side effects.","BTK inhibitors give durable responses in relapsed disease with zanubrutinib's approval in 2021."],"history":[{"year":1983,"title":"Isaacson and Wright describe MALT lymphoma","refs":[]},{"year":1993,"title":"H. pylori eradication induces regression of gastric MALT lymphoma (Wotherspoon)","refs":[]},{"year":2021,"title":"Zanubrutinib approved for relapsed marginal zone lymphoma","refs":["zanubrutinib"]}],"pipeline":["zanubrutinib","lenalidomide"],"openProblems":["Transformation to diffuse large B-cell lymphoma in a minority.","No standard sequence of therapies is proven by randomised trials.","Rare sites (lung, skin, dura) are managed by extrapolation."],"parent":"non-hodgkin-lymphoma"},"route":"/cancers/marginal-zone-lymphoma/","neighbours":{"drug":[{"id":"bendamustine","kind":"drug","name":"Bendamustine","route":"/drugs/bendamustine/"},{"id":"chlorambucil","kind":"drug","name":"Chlorambucil","route":"/drugs/chlorambucil/"},{"id":"ibrutinib","kind":"drug","name":"Ibrutinib","route":"/drugs/ibrutinib/"},{"id":"lenalidomide","kind":"drug","name":"Lenalidomide","route":"/drugs/lenalidomide/"},{"id":"rituximab","kind":"drug","name":"Rituximab","route":"/drugs/rituximab/"},{"id":"zanubrutinib","kind":"drug","name":"Zanubrutinib","route":"/drugs/zanubrutinib/"}],"cancer":[{"id":"non-hodgkin-lymphoma","kind":"cancer","name":"Non-Hodgkin lymphoma (all types)","route":"/cancers/non-hodgkin-lymphoma/"}],"technology":[{"id":"hypofractionated-radiotherapy","kind":"technology","name":"Hypofractionated radiotherapy","route":"/technologies/hypofractionated-radiotherapy/"},{"id":"imrt-igrt","kind":"technology","name":"IMRT / IGRT (modern external beam)","route":"/technologies/imrt-igrt/"}]}}