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MALT lymphoma

Last reviewed: 26 Dec 2024
Last updated: 10 Jan 2025

Summary

Definition

History and exam

Key diagnostic factors

  • history of Helicobacter pylori gastritis
  • history of autoimmune disorder
  • age >60 years
  • dyspepsia
  • epigastric discomfort
  • skin lesions
  • red eye ± photophobia
  • conjunctival fornix mass
  • painless proptosis, motility disturbances of the eye, diplopia, ptosis, decreased vision
Full details

Other diagnostic factors

  • abdominal pain, nausea, diarrhea, malabsorption, vomiting
  • GI bleeding, perforation, and obstruction
  • fever
  • night sweats
  • weight loss
  • shortness of breath, hemoptysis, cough
  • salivary gland swelling
  • thyroid swelling
  • esophageal/tracheal obstruction (shortness of breath, dysphagia, tracheal deviation)
  • breast lump
  • focal neurologic deficits
  • lymphadenopathy
Full details

Risk factors

  • Helicobacter pylori infection
  • age >60 years
  • autoimmune disease
  • Chlamydia psittaci infection
  • Campylobacter jejuni infection
  • Borrelia burgdorferi infection
  • hepatitis C virus infection
  • hepatitis B virus infection
  • HIV infection
  • achromobacter xylosoxidans infection
Full details

Diagnostic tests

1st tests to order

  • CBC with differential
  • blood smear
  • comprehensive metabolic panel (including LFTs)
  • serum lactate dehydrogenase (LDH)
  • serum beta-2 microglobulin
  • upper GI endoscopy
  • biopsy of affected site
  • immunophenotyping
  • H pylori (histologic testing)
  • CT scan
Full details

Tests to consider

  • H pylori (stool antigen test)
  • H pylori (urea breath test)
  • H pylori (serology test)
  • PCR testing for infectious agents
  • bone marrow biopsy with aspirate
  • genetic studies
  • immunoglobulin gene rearrangement studies
  • MYD88 mutation testing
  • serum protein electrophoresis
  • 18F-fluorodeoxyglucose (FDG) PET/CT scan
  • endoscopic ultrasound of the stomach
  • lower GI endoscopy
  • MRI orbit, brain
  • mammography
  • breast ultrasound
  • MRI breast
  • thyroid ultrasound
  • bronchoscopy
  • hepatitis C serology
  • hepatitis B serology
  • HIV serology
  • multigated acquisition (MUGA) scan
  • echocardiography
Full details

Treatment algorithm

ACUTE

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

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

advanced gastric MALT lymphoma

localized nongastric MALT lymphoma

advanced nongastric MALT lymphoma

high-grade histologic transformation

Contributors

Authors

Christopher McNamara, MBBS (Hons), FRACP, FRCPA, FRCPath

Consultant Haematologist

Royal Free Hospital

London

UK

Disclosures

CM declares that he has no competing interests.

Acknowledgements

Dr Christopher McNamara wishes to gratefully acknowledge Dr Rahul Joshi, a previous contributor to this topic. RJ declared that he had no competing interests. Unfortunately, we have since been made aware that Dr Rahul Joshi is deceased.

Peer reviewers

Markus Raderer, MD

Professor of Medicine

Department of Internal Medicine I

Division of Oncology

Medical University Vienna

Austria

Disclosures

MR is an author of a number of references cited in this topic.

Alan Lichtin, MD

Staff Hematologist

Cleveland Clinic Taussig Cancer Center

Cleveland

OH

Disclosures

AL declares that he has no competing interests.

Ian Chau, MD

Consultant Medical Oncologist

Royal Marsden Hospital

Sutton

UK

Disclosures

IC has been reimbursed by Roche Products, the manufacturer of rituximab, for attending several conferences. IC has also served on advisory boards and received honorarium for giving presentations for Roche Products. IC has received research funding from Novartis to conduct academic studies in the treatment of colorectal cancer.

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