Synonym: Mediterranean abdominal lymphoma
Epidemiologic features:
(1) It usually occurs in patients from the Middle East and areas around the Mediterranean Sea (Israel, Egypt, North Africa, Saudi Arabia).
(2) Patients usually have a low socioeconomic status.
Clinical features:
(1) The disease usually affects young adults (20's or 30's).
(2) The patient may present with:
(2a) diarrhea, steatorrhea, malabsorption
(2b) fever
(2c) abdominal pain
(2d) wasting
(2e) hypocalcemia
(3) The patient may have a history of Campylobacter jejuni infections and may reverse with antibiotic therapy.
Laboratory features:
(1) There is a monoclonal increase of IgA heavy chain in the serum.
(2) The tumor is a lymphoplasmacytic extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT, or GALT for gastrointestinal associated lymphoid tissue). It usually involves the small intestine and the mesenteric lymph nodes.
(3) The lymphoma cells stain with immunoperoxidase stains to IgA.
(4) The tumor may progress to a high grade large B-cell lymphoma.
