MALT lymphoma

Overview 
Theory 
Diagnosis 
Management 
Follow up 
Resources 

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ACUTE

localized gastric MALT lymphoma: Helicobacter pylori-positive and t(11;18)-negative (or unknown)

1st line – H pylori eradication therapy

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1st line –

H pylori eradication therapy

H pylori eradication therapy is the recommended initial treatment for patients with localized gastric mucosa-associated lymphoid tissue (MALT) lymphoma (Modified Blackledge stage I1, I2, and II1) who are H pylori-positive and t(11;18)-negative (or unknown).[4][23][47]

See Gastritis for information on H pylori eradication therapy regimens.

Eradication of H pylori should be assessed using a stool antigen test or urea breath test at least 6 weeks after starting eradication therapy and at least 2 weeks after stopping proton-pump inhibitor.[4][14][48] Patients who remain H pylori-positive after a first-line regimen should be treated with an alternative regimen.

Endoscopy and biopsy should be carried out at 3-6 months after eradication therapy to confirm H pylori eradication (histologically) and to evaluate lymphoma remission.[4][14][23] Lymphoma remission rate following H pylori eradication is approximately 70% to 80% in patients with localized gastric MALT lymphoma.[49][50] Time to remission following H pylori eradication can vary; therefore, continued observation with repeat endoscopy and biopsy may be required if treatment response is slow.[23][25]

Patients with complete response to eradication therapy at initial evaluation (i.e., negative for H pylori and lymphoma) should undergo long-term clinical follow-up (including endoscopy) to monitor for recurrence and histologic transformation.[4][14][23] See Monitoring section.

localized gastric MALT lymphoma: Helicobacter pylori-negative, or Helicobacter pylori-positive and t(11;18)-positive

1st line – radiation therapy

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1st line –

radiation therapy

Involved site radiation therapy (to the stomach and perigastric lymph node) can be used for initial treatment for patients with localized gastric mucosa-associated lymphoid tissue (MALT) lymphoma (Modified Blackledge stage I1, I2, and II1) who are H pylori-negative, or H pylori-positive and t(11;18)-positive.[4][14][23]

H pylori-negative patients, and H pylori-positive patients who are t(11;18)-positive are unlikely to respond to H pylori eradication therapy.[25][26]

Analysis of pooled data found that radiation therapy alone in patients unresponsive to H pylori eradication therapy achieves significantly higher remission rates compared with chemotherapy (97.3% vs. 85.3%), and similar remission rates compared with surgery (97.3% vs. 92.5%).[51]

Endoscopy and biopsy should be carried out to evaluate lymphoma remission at 3-6 months following radiation therapy.[4][14][23] Time to remission may vary following treatment; therefore, continued observation with repeat endoscopy and biopsy may be required if treatment response is slow.[23]

Patients who respond to radiation therapy (i.e., negative for lymphoma) should undergo long-term clinical follow-up (including endoscopy) to monitor for recurrence and histologic transformation.[4][14][23] See Monitoring section.

Consider – H pylori eradication therapy

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Consider –

H pylori eradication therapy

Treatment recommended for SOME patients in selected patient group

Patients who are H pylori-positive and t(11;18)-positive are unlikely to respond to H pylori eradication therapy.[25][26] However, H pylori eradication therapy can be given to these patients to treat the underlying H pylori infection.

See Gastritis for information on H pylori eradication therapy regimens.

2nd line – rituximab

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2nd line –

rituximab

Patients with localized gastric mucosa-associated lymphoid tissue (MALT) lymphoma (Modified Blackledge stage I1, I2, and II1) who are H pylori-negative, or H pylori-positive and t(11;18)-positive can be treated with rituximab (an anti-CD20 monoclonal antibody) if radiation therapy is contraindicated.[23]

One prospective study of 27 gastric MALT lymphoma patients who were resistant to, refractory to, or not suitable for H pylori eradication therapy reported an overall response rate of 77% following treatment with rituximab.[52]

In a subsequent retrospective study of rituximab-treated patients (n=28) with persistent or H pylori-negative disease, overall response rate was 73% and 5-year progression-free survival was 70%.[53]

Endoscopy and biopsy should be carried out to evaluate lymphoma remission at 3-6 months following treatment with rituximab.[4][14][23] Time to remission may vary following treatment; therefore, continued observation with repeat endoscopy and biopsy may be required if treatment response is slow.[23]

Patients who respond to rituximab (i.e., negative for lymphoma) should undergo long-term clinical follow-up (including endoscopy) to monitor for recurrence and histologic transformation.[4][14][23] See Monitoring section.

See local specialist protocol for dosing guidelines.

Primary options

rituximab

Consider – H pylori eradication therapy

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Consider –

H pylori eradication therapy

Treatment recommended for SOME patients in selected patient group

See Gastritis for information on H pylori eradication therapy regimens.

advanced gastric MALT lymphoma

1st line – systemic treatment or radiation therapy or observation

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1st line –

systemic treatment or radiation therapy or observation

Patients with advanced gastric mucosa-associated lymphoid tissue (MALT) lymphoma (Modified Blackledge stage II2, IIE, and IV) should be considered for systemic therapy or palliative involved site radiation therapy if they have indications for treatment, which include: symptoms; threatened end-organ function; clinically significant or progressive cytopenia secondary to lymphoma; clinically significant bulky disease; or steady or rapid progression.[23]

First-line systemic treatments include: chemoimmunotherapy (e.g., bendamustine plus rituximab; rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone [R-CHOP]; rituximab plus cyclophosphamide, vincristine, and prednisone [R-CVP]) or immunotherapy (e.g., lenalidomide plus rituximab; rituximab alone).[4][14][23]

The choice of systemic treatment should be individualized based on patient factors (e.g., age, performance status) and goal of therapy (e.g., disease control).

Patients can be observed (watch and wait) if they do not have any indications for treatment.

Patients should undergo long-term clinical follow-up to monitor for recurrence, disease progression, and histologic transformation.[4][14][23] See Monitoring section.

See local specialist protocol for dosing guidelines.

Primary options

bendamustine

and

rituximab

R-CHOP

rituximab

and

cyclophosphamide

and

doxorubicin

and

vincristine

and

prednisone

R-CVP

rituximab

and

cyclophosphamide

and

vincristine

and

prednisone

lenalidomide

and

rituximab

Consider – H pylori eradication therapy

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Consider –

H pylori eradication therapy

Treatment recommended for SOME patients in selected patient group

Helicobacter pylori eradication therapy can be considered in patients with advanced gastric MALT lymphoma who are H pylori-positive.[4][14][54]

See Gastritis for information on H pylori eradication therapy regimens.

localized nongastric MALT lymphoma

salivary gland

1st line – radiation therapy

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1st line –

radiation therapy

Involved site radiation therapy is recommended for treatment of localized nongastric mucosa-associated lymphoid tissue (MALT) lymphoma (Lugano stage IE and contiguous IIE) affecting the salivary gland.[4][14][23]

Xerostomia is a potential adverse effect of radiation therapy to the salivary gland.

Patients should undergo long-term clinical follow-up to monitor for recurrence, disease progression, and histologic transformation.[4][14][23] See Monitoring section.

2nd line – observation or rituximab

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2nd line –

observation or rituximab

Observation or rituximab can be considered in selected patients with localized nongastric MALT lymphoma affecting the salivary gland (e.g., if radiation therapy is likely to cause significant morbidity).[23]

Patients should undergo long-term clinical follow-up to monitor for recurrence, disease progression, and histologic transformation.[4][14][23] See Monitoring section.

See local specialist protocol for dosing guidelines.

Primary options

rituximab

ocular adnexa

1st line – radiation therapy

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1st line –

radiation therapy

Adverse effects associated with radiation therapy to the ocular adnexa are dose-dependent and include cataract and dry eye.[14][23] Late effects of high-dose involved site radiation therapy include retinopathy, optic atrophy, corneal ulceration, and glaucoma.[55]

Patients should undergo long-term clinical follow-up to monitor for recurrence, disease progression, and histologic transformation.[4][14][23] See Monitoring section.

Consider – antibiotic therapy

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Consider –

antibiotic therapy

Treatment recommended for SOME patients in selected patient group

Chlamydia psittaci eradication with doxycycline can be considered if C psittaci is detected in the biopsy specimen.[4][14][56]

Primary options

doxycycline: 100 mg orally twice daily for 21 days

2nd line – observation or rituximab

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2nd line –

observation or rituximab

Observation or rituximab can be considered in selected patients with localized nongastric MALT lymphoma affecting the ocular adnexa (e.g., if radiation therapy is likely to cause significant morbidity).[23]

Patients should undergo long-term clinical follow-up to monitor for recurrence, disease progression, and histologic transformation.[4][14][23] See Monitoring section.

See local specialist protocol for dosing guidelines.

Primary options

rituximab

Consider – antibiotic therapy

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Consider –

antibiotic therapy

Treatment recommended for SOME patients in selected patient group

Chlamydia psittaci eradication with doxycycline can be considered if C psittaci is detected in the biopsy specimen.[4][14][56]

Primary options

doxycycline: 100 mg orally twice daily for 21 days

lung

1st line – radiation therapy or surgery

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1st line –

radiation therapy or surgery

Involved site radiation therapy or surgery (limited resection) is recommended for treatment of localized nongastric mucosa-associated lymphoid tissue (MALT) lymphoma (Lugano stage IE and contiguous IIE) affecting the lung.[23][57][58][59]

Radiation therapy can cause acute phase adverse effects (e.g., pneumonitis) and long-term morbidity; consider lower-dose radiation therapy.[60]

Patients should undergo long-term clinical follow-up to monitor for recurrence, disease progression, and histologic transformation.[4][14][23] See Monitoring section.

2nd line – observation or rituximab

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2nd line –

observation or rituximab

Observation or rituximab can be considered in selected patients with localized nongastric MALT lymphoma affecting the lung (e.g., if radiation therapy is likely to cause significant morbidity).[23]

Patients should undergo long-term clinical follow-up to monitor for recurrence, disease progression, and histologic transformation.[4][14][23] See Monitoring section.

See local specialist protocol for dosing guidelines.

Primary options

rituximab

skin (solitary or regional disease)

1st line – radiation therapy or surgery or observation

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1st line –

radiation therapy or surgery or observation

Involved site radiation therapy is the preferred initial treatment for localized (solitary or regional) cutaneous disease.[46][61]

Surgical excision is also an option in selected patients.[46]

Observation is recommended if radiation therapy or surgery is not feasible.[46]

Patients should undergo long-term clinical follow-up to monitor for recurrence, disease progression, and histologic transformation.[4][14][23] See Monitoring section.

skin (generalized disease)

1st line – observation

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1st line –

observation

Observation is recommended for patients with multiple cutaneous lesions (generalized disease) who are asymptomatic.[46]

Patients should undergo long-term clinical follow-up to monitor for disease progression and histologic transformation.[4][14][23] See Monitoring section.

Consider – radiation therapy or rituximab

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Consider –

radiation therapy or rituximab

Treatment recommended for SOME patients in selected patient group

Palliative radiation therapy or rituximab can be used to treat symptomatic cutaneous lesions in patients with generalized disease.[14][46]

See local specialist protocol for dosing guidelines.

Primary options

rituximab

thyroid, breast, or dura

1st line – radiation therapy or surgery

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1st line –

radiation therapy or surgery

Surgical excision may be considered for localized disease affecting the thyroid or breast.[23]

Patients should undergo long-term clinical follow-up to monitor for recurrence, disease progression, and histologic transformation.[4][14][23] See Monitoring section.

2nd line – observation or rituximab

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2nd line –

observation or rituximab

Observation or rituximab can be considered in selected patients with localized nongastric mucosa-associated lymphoid tissue (MALT) lymphoma affecting the thyroid, breast, or dura (e.g., if radiation therapy is likely to cause significant morbidity).[23]

Patients should undergo long-term clinical follow-up to monitor for recurrence, disease progression, and histologic transformation.[4][14][23] See Monitoring section.

See local specialist protocol for dosing guidelines.

Primary options

rituximab

advanced nongastric MALT lymphoma

1st line – radiation therapy or observation

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1st line –

radiation therapy or observation

Involved site radiation therapy is recommended for patients with advanced nongastric (noncutaneous) mucosa-associated lymphoid tissue (MALT) lymphoma (i.e., Lugano stage IV).[23]

Observation can be considered in selected patients (e.g., if radiation therapy is likely to cause significant morbidity).[23]

Patients should undergo long-term clinical follow-up to monitor for recurrence, disease progression, and histologic transformation.[4][14][23] See Monitoring section.

high-grade histologic transformation

1st line – chemoimmunotherapy

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1st line –

chemoimmunotherapy

Patients with high-grade histologic transformation to diffuse large B-cell lymphoma (DLBCL) should be managed according to this disease.

See Non-Hodgkin lymphoma.

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MALT lymphoma

Treatment algorithm

localized gastric MALT lymphoma: Helicobacter pylori-positive and t(11;18)-negative (or unknown)

H pylori eradication therapy

localized gastric MALT lymphoma: Helicobacter pylori-negative, or Helicobacter pylori-positive and t(11;18)-positive

radiation therapy

H pylori eradication therapy

rituximab

Primary options

H pylori eradication therapy

advanced gastric MALT lymphoma

systemic treatment or radiation therapy or observation

Primary options

R-CHOP

R-CVP

H pylori eradication therapy

localized nongastric MALT lymphoma

radiation therapy

observation or rituximab

Primary options

radiation therapy

antibiotic therapy

Primary options

observation or rituximab

Primary options

antibiotic therapy

Primary options

radiation therapy or surgery

observation or rituximab

Primary options

radiation therapy or surgery or observation

observation

radiation therapy or rituximab

Primary options

radiation therapy or surgery

observation or rituximab

Primary options

advanced nongastric MALT lymphoma

radiation therapy or observation

high-grade histologic transformation

chemoimmunotherapy