History and exam

Key diagnostic factors

common

history of Helicobacter pylori gastritis

Strongly associated with gastric MALT lymphoma (detected in approximately 90% of cases).[15]​​[16]​​​

Also associated with lung and liver MALT lymphoma.​[14]

Presence may be known at time of presentation or only discovered during work-up.

history of autoimmune disorder

There is a strong association between autoimmune disease and non-gastric MALT lymphoma.

Salivary/parotid gland MALT lymphoma is associated with lymphoepithelial sialadenitis and Sjögren's syndrome.[14] Thyroid MALT lymphoma is associated with Hashimoto's thyroiditis.[14] Lung MALT lymphoma is associated with Sjögren's syndrome, systemic lupus erythematosus, and common variable immunodeficiency syndrome.​[14]

age >60 years

In the US, median age at diagnosis of MALT lymphoma is approximately 67 years.[12]​​ 

dyspepsia

A common presenting symptom of gastric MALT lymphoma.

An association between gastric MALT lymphoma and chronic gastritis secondary to H pylori infection is reported in up to 90% of cases.[15][16]​​

epigastric discomfort

A common presenting symptom of gastric MALT lymphoma.

An association between gastric MALT lymphoma and chronic gastritis secondary to H pylori infection is reported in up to 90% of cases.[15]​​[16]​​

uncommon

skin lesions

Cutaneous MALT lymphoma presents as single or multiple papulonodular lesions or plaques that are brown or reddish-brown in colour and are mainly seen over the limbs or back.

Skin/cutaneous MALT lymphoma is associated with Borrelia burgdorferi infection.[14]

red eye ± photophobia

Ocular adnexa MALT lymphoma presents as painless conjunctival injection (red eye) with or without photophobia. This mimics allergic conjunctivitis.

conjunctival fornix mass

In patients with ocular adnexa MALT lymphoma, physical examination may reveal orange or salmon-pink masses in the conjunctival fornices.

painless proptosis, motility disturbances of the eye, diplopia, ptosis, decreased vision

Can occur if the orbit is involved. Orbital MALT lymphomas are more common in the anterior superior orbit.

Other diagnostic factors

uncommon

abdominal pain, nausea, diarrhoea, malabsorption, vomiting

Rare presenting signs and symptoms of gastric MALT lymphoma. Due to gastritis.

An association between gastric MALT lymphoma and chronic gastritis secondary to H pylori infection is reported in up to 90% of cases.[15]​​[16]​​

GI bleeding, perforation, and obstruction

Described in cases of gastric MALT lymphoma, but occurs infrequently.

Usually due to the association between gastric MALT lymphoma and chronic gastritis secondary to H pylori infection.

fever

B symptoms (unexplained fevers [>38ºC (>100ºF)]; drenching night sweats requiring a change of clothing; and significant weight loss [>10% of body weight in <6 months]) are rare in gastric MALT lymphoma.

Fever may occur in lung MALT lymphoma.[9]

night sweats

B symptoms (unexplained fevers [>38ºC (>100ºF)]; drenching night sweats requiring a change of clothing; and significant weight loss [>10% of body weight in <6 months]) are rare in gastric MALT lymphoma.

weight loss

B symptoms (unexplained fevers [>38ºC (>100ºF)]; drenching night sweats requiring a change of clothing; and significant weight loss [>10% of body weight in <6 months]) are rare in gastric MALT lymphoma.

Weight loss may occur in lung MALT lymphoma.[9]

shortness of breath, haemoptysis, cough

Lung MALT lymphoma may present with dyspnoea, haemoptysis, and cough.[9]

salivary gland swelling

Salivary/parotid gland MALT lymphoma may present with an enlarging salivary gland mass.

thyroid swelling

Thyroid MALT lymphoma may present with a thyroid mass.

oesophageal/tracheal obstruction (shortness of breath, dysphagia, tracheal deviation)

Thyroid MALT lymphoma may present with signs and symptoms of oesophageal/tracheal obstruction (dysponea, dysphagia, tracheal deviation) if the thyroid mass is large.

breast lump

Breast MALT lymphoma may present as a painless enlarging breast mass.

focal neurological deficits

Due to dural involvement.

Deficit depends on the specific location of the lymphoma.

lymphadenopathy

An uncommon finding in gastric MALT lymphoma.[30]

Risk factors

strong

Helicobacter pylori infection

Gastric MALT lymphoma is strongly associated with chronic Helicobacter pylori infection, which is detected in up to 90% of cases.[15]​​[16]​​

Lung and liver MALT lymphoma is also associated with infection with H pylori.[14]​​

age >60 years

In the US, median age at diagnosis of MALT lymphoma is approximately 67 years.[12]​​

autoimmune disease

There is a strong association between autoimmune disease and non-gastric MALT lymphoma.

Salivary/parotid gland MALT lymphoma is associated with lymphoepithelial sialadenitis and Sjögren's syndrome.[14]​​ Thyroid MALT lymphoma is associated with Hashimoto's thyroiditis.[14] Lung MALT lymphoma is associated with Sjögren's syndrome, systemic lupus erythematosus, and common variable immunodeficiency syndrome.[14]

weak

Chlamydia psittaci infection

Associated with ocular adnexa MALT lymphoma.[14]

Campylobacter jejuni infection

Associated with intestinal MALT lymphoma (including immunoproliferative small intestinal disease).[14]

Borrelia burgdorferi infection

Associated with skin/cutaneous MALT lymphoma.[14]

hepatitis C virus infection

Hepatitis C virus infection is associated with MALT lymphoma of the salivary/parotid gland, lung, ocular adnexa, and liver.[14]

hepatitis B virus infection

Hepatitis B virus infection is associated with liver MALT lymphoma.[17]

HIV infection

Weak association with lung MALT lymphoma.[14]

achromobacter xylosoxidans infection

Associated with lung MALT lymphoma.[14]

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