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Home > Professional Reference > Mucosa-associated Lymphoid Tissue (MALT) Lymphoma

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Mucosa-associated Lymphoid Tissue (MALT) Lymphoma

This PatientPlus article is written for healthcare professionals so the language may be more technical than the condition leaflets. You may find the abbreviations list helpful.

The body's immune system is made up of a number of masses of lymphoid tissue or organs, as well as circulating leukocytes that originate from the bone marrow. The main lymphoid organs are:

  • Bone marrow, thymus, tonsils, spleen and lymph nodes
  • Mucosa-associated lymphoid tissue (MALT)
  • Gut-associated lymphoid tissue (GALT)
  • Bronchus-associated lymphoid tissue (BALT)
  • Skin-associated lymphoid tissue (SALT)

Definition

MALT lymphoma is a subtype of non-Hodgkin's lymphoma with it's own specific pathology, histology and clinical features.1 It is distinct because it involves lymphoid proliferation in mucosa-associated lymphoid tissue (MALT) rather than lymph nodes.

The distinction from other non-Hodgkin's lymphoma was first made by Isaacson and Wright in 1983.2 It was described as a discrete form of lymphoma in 1994 in the Revised European-American Lymphoma (REAL) classification.1 In this classification, it is classified as one of the subtypes of marginal zone B-cell lymphomas which include nodal, extranodal (MALT-type) and splenic.

MALT lymphomas follow a different course to nodal B-cell lymphomas. They tend to remain localised for longer, lack poor prognostic features and have a higher 5-year survival rate.3 MALT lymphomas can be divided into:

  • Gastric: the most common type and associated with Helicobacter pylori infection.
  • Non-gastric: most often in the head and neck, lung and eye. Non-gastric MALT lymphomas are not associated with H. pylori infection.

Aetiology

  • Mucosa-associated lymphoid tissue (MALT) can develop in nearly every organ as a result of chronic infection or an autoimmune process. If there is prolonged lymphoid proliferation, a malignant clone can emerge and a MALT lymphoma could follow.
  • Certain infections have been associated with MALT lymphomas:
  • Autoimmune diseases such as Hashimoto's thyroiditis and Sjögren's syndrome have also been linked to MALT lymphomas in the thyroid and salivary glands.
  • There are certain karyotypic abnormalities associated with MALT lymphoma, the most common being the translocation t(11;18)(q21;q21) found in one third of cases. Trisomy 3 and trisomy 18 are other abnormalities.11,12,13 These abnormalities seem to drive lymphoma progression.

Epidemiology

  • Mucosa-associated lymphoid tissue (MALT) lymphoma accounts for 8% of all non-Hodgkin's lymphomas.14
  • It is most likely to present in the sixth decade.1
  • Females are affected more than males.1
  • Gastric MALT lymphoma is the most common.14

Clinical features

  • These depend on the site involved:
    • Gastric mucosa-associated lymphoid tissue (MALT) lymphomas may present with dyspepsia.
    • Nonspecific symptoms include fatigue, low-grade fever, nausea, constipation, weight loss and anaemia.
    • Recurrent respiratory tract infections.
    • Orbital MALT lymphomas may present with blurred vision and visual field defects.
  • MALT lymphoma usually follows an indolent course. It can remain localised for long periods.
  • Disseminated disease is more likely in non-gastrointestinal MALT lymphoma15,16 but there is still a 5-year survival rate of 93% for this subtype.14
  • Rarely, there is transformation to a more aggressive form of lymphoma.
  • Relapses of MALT lymphoma may be late and lifelong observation may be required.17

Investigations

  • General assessment: full blood count, renal function tests, electrolytes, liver function tests
  • Phenotyping of circulating lymphocytes, bone marrow lymphocytes or biopsy specimens
  • Imaging studies for disease staging:
    • Barium contrast studies of the upper and lower gastrointestinal tract
    • CT scan and MRI scan
  • Endoscopy
  • Bone marrow aspiration

Staging

  • Stage IE: lymphoma is present in only 1 area or organ outside the lymph nodes.
  • Stage IIE: lymphoma is present in only 1 area or organ outside the lymph nodes and in the lymph nodes around it.
  • Stage IIIE: lymphoma is present on both sides of the diaphragm. It may also have spread to an area or organ near the lymph nodes and/or the spleen.
  • Stage IV: lymphoma is widespread to several organs, with or without lymph node involvement.

E designates that all MALT lymphomas are extranodal in origin.

Management

Management is different for gastric and non-gastric mucosa-associated lymphoid tissue (MALT) lymphomas.

  • Treatment with a proton pump inhibitor (PPI) and antibiotics to eradicate H. pylori is the main treatment for most gastric MALT lymphomas, which are often low-grade and remain localised for several years. Treatment with chemotherapy, surgery or radiotherapy has not been demonstrated to be superior to antibiotic treatment.
  • Treatments for non-gastric MALT lymphomas include radiotherapy, chemotherapy, monoclonal antibodies and surgery.

Surgery has only a limited role in treatment. Surgery for non-gastrointestinal MALT lymphoma is usually restricted to excisional biopsies. Partial or total gastrectomy is associated with considerable morbidity and is rarely necessary.

Specific types of mucosa-associated lymphoid tissue (MALT) lymphoma

Gastric MALT lymphoma

  • Associated with: H. pylori infection.
  • Presenting features: dyspepsia, epigastric discomfort, gastric bleeding. Systemic symptoms (night sweats, weight loss, fever, lethargy) and bone marrow involvement are less common. Most are localised to the stomach at presentation.
  • Diagnosis: endoscopy and gastric biopsy. There are characteristic histological features.
  • Treatment:
    • Eradication of H. pylori with antibiotics and a proton pump inhibitor (PPI) or H2-receptor antagonist can lead to a complete remission of gastric MALT lymphoma in between 65-70% of cases and is first-line treatment for early-stage disease.1,18,19
    • If H. pylori status is negative, eradication treatment may not work.
    • Close follow-up using endoscopy is needed after eradication treatment to ensure a complete response.20
    • If there is locally-advanced or high-grade disease, chemotherapy, monoclonal antibody treatment with rituximab, or radiotherapy should be added to eradication treatment.
    • Surgery is reserved for refractory disease as gastric preservation is preferred if possible.
    • Current clinical trials involving these different treatment modalities are underway.
  • Prognosis: 5-year survival rates >80%.21 Worst prognosis if there is deep infiltration of the gastric wall at presentation as spread to regional lymph nodes is more likely.22

Salivary gland MALT lymphoma

  • Associated with: Sjögren's syndrome.
  • Clinical features: parotid gland is the most common site. Indolent course (3-18 years' progression even with no treatment).23
  • Treatment: no agreed optimum approach. Surgery, radiotherapy, chemotherapy and monoclonal antibody therapy are all used.

Ocular adnexa and lacrimal gland MALT lymphoma

  • Associated with: Chlamydophila psittaci infection.
  • Clinical features: painless conjunctival injection, photophobia, orange/salmon-pink masses in the fornices.
  • Treatment: radiotherapy. Cataract and dry eyes are complications of this.24
  • Prognosis: 5-year survival rate 91%.25

Lung MALT lymphoma

  • Associated with: autoimmune diseases (Sjögren's syndrome, rheumatoid arthritis).
  • Clinical features: arises from bronchus-associated lymphoid tissue (BALT). 40% are asymptomatic and present with a solitary pulmonary nodule on CXR.1 There may be cough, dyspnoea, haemoptysis, fever, weight loss. Can spread throughout the lung and to other MALT.
  • Treatment: surgery (if localised), chemotherapy, radiotherapy.

Thyroid MALT lymphoma

  • Associated with: autoimmune thyroiditis.
  • Clinical features: thyroid mass, possible obstructive symptoms.
  • Diagnosis: may need open biopsy.
  • Treatment: surgery ± radiation for local disease; chemotherapy added if the disease is advanced.

Skin MALT lymphoma

  • Associated with: Borrelia burgdorferi infection.
  • Clinical features: single or multiple brown papulonodular lesions or plaques on the back or extremities.26,27
  • Treatment: observation only, excision, radiotherapy. Possibly treatment by eradication of Borrelia burgdorferi.
  • Prognosis: indolent. 5-year survival >95%.1

Document references

  1. Cohen SM, Petryk M, Varma M, et al; Non-Hodgkin's lymphoma of mucosa-associated lymphoid tissue. Oncologist. 2006 Nov-Dec;11(10):1100-17. [abstract]
  2. Isaacson P, Wright DH; Malignant lymphoma of mucosa-associated lymphoid tissue. A distinctive type of B-cell lymphoma. Cancer. 1983 Oct 15;52(8):1410-6. [abstract]
  3. Nathwani BN, Anderson JR, Armitage JO, et al; Marginal zone B-cell lymphoma: A clinical comparison of nodal and mucosa-associated lymphoid tissue types. Non-Hodgkin's Lymphoma Classification Project. J Clin Oncol. 1999 Aug;17(8):2486-92. [abstract]
  4. Ferreri AJ, Guidoboni M, Ponzoni M, et al; Evidence for an association between Chlamydia psittaci and ocular adnexal lymphomas. J Natl Cancer Inst. 2004 Apr 21;96(8):586-94. [abstract]
  5. Cerroni L, Zochling N, Putz B, et al; Infection by Borrelia burgdorferi and cutaneous B-cell lymphoma. J Cutan Pathol. 1997 Sep;24(8):457-61. [abstract]
  6. Roggero E, Zucca E, Mainetti C, et al; Eradication of Borrelia burgdorferi infection in primary marginal zone B-cell lymphoma of the skin. Hum Pathol. 2000 Feb;31(2):263-8. [abstract]
  7. Lecuit M, Abachin E, Martin A, et al; Immunoproliferative small intestinal disease associated with Campylobacter jejuni. N Engl J Med. 2004 Jan 15;350(3):239-48. [abstract]
  8. Luppi M, Longo G, Ferrari MG, et al; Additional neoplasms and HCV infection in low-grade lymphoma of MALT type. Br J Haematol. 1996 Aug;94(2):373-5. [abstract]
  9. Ascoli V, Lo Coco F, Artini M, et al; Extranodal lymphomas associated with hepatitis C virus infection. Am J Clin Pathol. 1998 May;109(5):600-9. [abstract]
  10. Girard T, Luquet-Besson I, Baran-Marszak F, et al; HIV+ MALT lymphoma remission induced by highly active antiretroviral therapy alone. Eur J Haematol. 2005 Jan;74(1):70-2. [abstract]
  11. Levine EG, Arthur DC, Machnicki J, et al; Four new recurring translocations in non-Hodgkin lymphoma. Blood. 1989 Oct;74(5):1796-800. [abstract]
  12. Auer IA, Gascoyne RD, Connors JM, et al; t(11;18)(q21;q21) is the most common translocation in MALT lymphomas. Ann Oncol. 1997 Oct;8(10):979-85. [abstract]
  13. Wotherspoon AC, Finn TM, Isaacson PG; Trisomy 3 in low-grade B-cell lymphomas of mucosa-associated lymphoid tissue. Blood. 1995 Apr 15;85(8):2000-4. [abstract]
  14. Cavalli F, Isaacson PG, Gascoyne RD, et al; MALT Lymphomas. Hematology Am Soc Hematol Educ Program. 2001;:241-58. [abstract]
  15. Thieblemont C, Berger F, Dumontet C, et al; Mucosa-associated lymphoid tissue lymphoma is a disseminated disease in one third of 158 patients analyzed. Blood. 2000 Feb 1;95(3):802-6. [abstract]
  16. Zinzani PL, Magagnoli M, Galieni P, et al; Nongastrointestinal low-grade mucosa-associated lymphoid tissue lymphoma: analysis of 75 patients. J Clin Oncol. 1999 Apr;17(4):1254. [abstract]
  17. Raderer M, Streubel B, Woehrer S, et al; High relapse rate in patients with MALT lymphoma warrants lifelong follow-up. Clin Cancer Res. 2005 May 1;11(9):3349-52. [abstract]
  18. Thiede C, Morgner A, Alpen B, et al; What role does Helicobacter pylori eradication play in gastric MALT and gastric MALT lymphoma? Gastroenterology. 1997 Dec;113(6 Suppl):S61-4. [abstract]
  19. Nakamura S, Matsumoto T, Suekane H, et al; Long-term clinical outcome of Helicobacter pylori eradication for gastric mucosa-associated lymphoid tissue lymphoma with a reference to second-line treatment. Cancer. 2005 Aug 1;104(3):532-40. [abstract]
  20. Fischbach W, Goebeler-Kolve M, Ruskone-Fourmestraux A, et al; Most patients with minimal histological residuals of gastric MALT lymphoma after successful eradication of Helicobacter pylori can be safely managed by a watch-and-wait strategy. Experience from a large international series. Gut. 2007 Jul 16;. [abstract]
  21. Pinotti G, Zucca E, Roggero E, et al; Clinical features, treatment and outcome in a series of 93 patients with low-grade gastric MALT lymphoma. Leuk Lymphoma. 1997 Aug;26(5-6):527-37. [abstract]
  22. Eidt S, Stolte M, Fischer R; Factors influencing lymph node infiltration in primary gastric malignant lymphoma of the mucosa-associated lymphoid tissue. Pathol Res Pract. 1994 Nov;190(11):1077-81. [abstract]
  23. Diss TC, Wotherspoon AC, Speight P, et al; B-cell monoclonality, Epstein Barr virus, and t(14;18) in myoepithelial sialadenitis and low-grade B-cell MALT lymphoma of the parotid gland. Am J Surg Pathol. 1995 May;19(5):531-6. [abstract]
  24. Tanimoto K, Kaneko A, Suzuki S, et al; Long-term follow-up results of no initial therapy for ocular adnexal MALT lymphoma. Ann Oncol. 2006 Jan;17(1):135-40. Epub 2005 Oct 19. [abstract]
  25. Uno T, Isobe K, Shikama N, et al; Radiotherapy for extranodal, marginal zone, B-cell lymphoma of mucosa-associated lymphoid tissue originating in the ocular adnexa: a multiinstitutional, retrospective review of 50 patients. Cancer. 2003 Aug 15;98(4):865-71. [abstract]
  26. Rijlaarsdam JU, van der Putte SC, Berti E, et al; Cutaneous immunocytomas: a clinicopathologic study of 26 cases. Histopathology. 1993 Aug;23(2):117-25. [abstract]
  27. Cerroni L, Signoretti S, Hofler G, et al; Primary cutaneous marginal zone B-cell lymphoma: a recently described entity of low-grade malignant cutaneous B-cell lymphoma. Am J Surg Pathol. 1997 Nov;21(11):1307-15. [abstract]

Acknowledgements

EMIS is grateful to Dr Colin Tidy for writing this article and to Dr Michelle Wright for earlier versions. The final copy has passed scrutiny by the independent Mentor GP reviewing team. ©EMIS 2010.
Document ID: 7089
Document Version: 3
Document Reference: bgp26098
Last Updated: 25 May 2010
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