# Marginal zone lymphoma

Source: https://onco.cc/cancers/marginal-zone-lymphoma/  
OnCo record `marginal-zone-lymphoma` (Cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

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.

## Fields

- Kind: Cancer
- Last checked: 2026-09-17
- Also known as: MZL; MALT lymphoma; Extranodal marginal zone lymphoma; Splenic marginal zone lymphoma; Nodal marginal zone lymphoma
- Tags: subtype-page
- 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

## Standard of care

- 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)](https://onco.cc/cancers/non-hodgkin-lymphoma/))
- 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)](https://onco.cc/technologies/imrt-igrt/), [Hypofractionated radiotherapy](https://onco.cc/technologies/hypofractionated-radiotherapy/))
- Splenic marginal zone lymphoma: Watch and wait if asymptomatic; antiviral therapy if hepatitis C-positive; rituximab alone or with chemotherapy; splenectomy now rare. ([Rituximab](https://onco.cc/drugs/rituximab/))
- Advanced or relapsed: Rituximab with bendamustine or chlorambucil, lenalidomide-rituximab; zanubrutinib or ibrutinib for relapsed disease. ([Rituximab](https://onco.cc/drugs/rituximab/), [Bendamustine](https://onco.cc/drugs/bendamustine/), [Chlorambucil](https://onco.cc/drugs/chlorambucil/), [Lenalidomide](https://onco.cc/drugs/lenalidomide/), [Zanubrutinib](https://onco.cc/drugs/zanubrutinib/), [Ibrutinib](https://onco.cc/drugs/ibrutinib/))

## State of the art

- 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.

## Open problems

- 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.

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Marginal_zone_B-cell_lymphoma
- Wikipedia: https://en.wikipedia.org/wiki/Marginal_zone_B-cell_lymphoma

## Connected records

- drugs: [Bendamustine](https://onco.cc/drugs/bendamustine/), [Chlorambucil](https://onco.cc/drugs/chlorambucil/), [Ibrutinib](https://onco.cc/drugs/ibrutinib/), [Lenalidomide](https://onco.cc/drugs/lenalidomide/), [Rituximab](https://onco.cc/drugs/rituximab/), [Zanubrutinib](https://onco.cc/drugs/zanubrutinib/)
- cancers: [Non-Hodgkin lymphoma (all types)](https://onco.cc/cancers/non-hodgkin-lymphoma/)
- technologies: [Hypofractionated radiotherapy](https://onco.cc/technologies/hypofractionated-radiotherapy/), [IMRT / IGRT (modern external beam)](https://onco.cc/technologies/imrt-igrt/)

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