Last reviewed: June 2020
Last updated: December  2017



History and exam

Key diagnostic factors

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

Other diagnostic factors

  • nausea and/or vomiting
  • GI bleeding
  • fever
  • night sweats
  • weight loss
  • shortness of breath, hemoptysis, cough
  • salivary gland swelling
  • thyroid swelling
  • breast lump
  • focal neurologic deficits
  • lymphadenopathy

Risk factors

  • Helicobacter pylori infection
  • age >60 years
  • autoimmune diseases
  • female gender
  • Chlamydia psittaci infection
  • Campylobacter jejuni infection
  • Borrelia burgdorferi infection
  • hepatitis C virus infection
  • HIV infection

Diagnostic investigations

1st investigations to order

  • CBC
  • blood smear
  • upper GI endoscopy
  • biopsy of affected tissue
  • H pylori serology
  • H pylori stool antigen test
  • LFTs
  • basic metabolic panel
More 1st investigations to order

Investigations to consider

  • bone marrow biopsy
  • immunohistochemistry
  • reverse transcriptase polymerase chain reaction (RT-PCR) or fluorescence in situ hybridization (FISH)
  • immunoglubulin gene rearrangement studies
  • serum electrophoresis
  • serum LDH
  • serum beta-2 microglobulin
  • staging CT scan
  • endoscopic ultrasound of the stomach
  • double contrast radiograph of the GI tract
  • lower GI endoscopy
  • endoscopic otolaryngology
  • CT scan of salivary glands
  • MRI orbit
  • hepatitis C serology
  • hepatitis B serology
  • HIV serology
  • multiple-gated acquisition scan (MUGA)
  • echocardiography
  • ethylenediamine tetraacetic acid glomerular filtration rate (EDTA GFR)
More investigations to consider

Treatment algorithm


Consultant Haematologist

Luton and Dunstable Hospital NHS Foundation Trust




RJ declares that he has no competing interests.

Consultant Haematologist

Royal Free Hospital




CM declares that he has no competing interests.

Peer reviewersVIEW ALL

Professor of Medicine

Department of Internal Medicine I

Division of Oncology

Medical University Vienna



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

Staff Hematologist

Cleveland Clinic Taussig Cancer Center




AL declares that he has no competing interests.

Consultant Medical Oncologist

Royal Marsden Hospital




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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