Approach

Treatment for mucosa-associated lymphoid tissue (MALT) lymphoma is based on the affected site (gastric or nongastric), disease stage (localized or advanced), and symptoms. Other factors that guide treatment include infection (e.g., Helicobacter pylori, Chlamydia psittaci) and genetic abnormalities (e.g., t(11;18)).

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

H pylori eradication therapy is the recommended initial treatment for patients with localized gastric 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.

Treatment evaluation and follow-up (after eradication therapy)

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 the proton pump inhibitor (PPI).[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: H pylori-negative, or H pylori-positive and t(11;18)-positive

Involved site radiation therapy (to the stomach and perigastric lymph node) can be used for initial treatment for patients with localized gastric MALT lymphoma (Modified Blackledge stage I1, I2, and II1) who are:[4][14][23]

  • H pylori-negative

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

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 to surgery (97.3% vs. 92.5%).[51]

Patients not suitable for radiation therapy

If radiation therapy is contraindicated, patients can be treated with rituximab (an anti-CD20 monoclonal antibody).[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]

Treatment evaluation and follow-up (after radiation therapy or rituximab)

Endoscopy and biopsy should be carried out to evaluate lymphoma remission at 3-6 months following treatment with radiation therapy or 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 radiation therapy or 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.​

Advanced gastric MALT lymphoma

Patients with advanced gastric 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:[23]

  • Symptoms

  • Threatened end-organ function

  • Clinically significant or progressive cytopenia secondary to lymphoma

  • Clinically significant bulky disease

  • Steady or rapid progression

 First-line systemic treatments include:[4][14][23]

  • Chemoimmunotherapy (e.g., bendamustine plus rituximab; rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone [R-CHOP]; rituximab plus cyclophosphamide, vincristine, and prednisone [R-CVP])

  • Immunotherapy (e.g., lenalidomide plus rituximab; rituximab alone)

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.[23] 

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

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

Localized nongastric MALT lymphoma

Treatment for localized nongastric MALT lymphoma (e.g., Lugano stage IE and contiguous IIE for noncutaneous sites) is based on the affected nongastric site (e.g., salivary gland, ocular adnexa, lung, skin, thyroid, breast, dura).

Salivary gland

  • Involved site radiation therapy is recommended for treatment of localized disease affecting the salivary gland.[4][14][23] Observation or rituximab can be considered in selected patients (e.g., if radiation therapy is likely to cause significant morbidity).[23]

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

Ocular adnexa

  • Involved site radiation therapy is recommended for treatment of localized disease affecting the ocular adnexa.[4][14][23] Observation or rituximab can be considered in selected patients (e.g., if radiation therapy is likely to cause significant morbidity).[23]

  • Adverse effects associated with radiation therapy to the ocular adnexa are dose-dependent and include cataract and dry eye.[4][14] Late effects of high-dose involved site radiation therapy include retinopathy, optic atrophy, corneal ulceration, and glaucoma.[55]

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

Lung

  • Involved site radiation therapy or surgery (limited resection) is recommended for treatment of localized disease affecting the lung.[23][57][58][59] Observation or rituximab can be considered in selected patients (e.g., if radiation therapy is likely to cause significant morbidity).[23] 

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

Skin

  • 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, or if patients have multiple cutaneous lesions (generalized disease) and are asymptomatic.[46] Palliative radiation therapy or rituximab can be used to treat symptomatic cutaneous lesions in patients with generalized disease.[14][46]

  • There are case reports of complete regression of cutaneous MALT lymphoma following antibiotic treatment of associated Borrelia burgdorferi infection, but this is not standard practice.[14][62]​​

Thyroid, breast, or dura

  • Involved site radiation therapy is recommended for treatment of localized disease affecting the thyroid, breast, or dura.[14][23]​​ Surgical excision may be considered for localized disease affecting the thyroid or breast.[23] Observation or rituximab can be considered in selected patients (e.g., if radiation therapy is likely to cause significant morbidity).[23] 

Treatment evaluation and follow-up

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

Advanced nongastric MALT lymphoma

Involved site radiation therapy is recommended for patients with advanced nongastric (noncutaneous) 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 relapse, disease progression, and histologic transformation.[4][14][23] See Monitoring section.

High-grade histologic transformation

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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