Immunoproliferative Small Intestinal Disease And Small Bowel Lymphoma

Immunoproliferative Small Intestinal Disease And Small Bowel Lymphoma

Immunoproliferative Small Intestinal Disease is five times  more common in males than in females and mainly occurs in the aged 50 to 70 years.

IPSID and small bowel lymphoma, also termed Mediterranean lymphoma, is not uncommon in the tropics, and usually affects socioeconomically disadvantaged persons.

IPSID presentation is  with chronic diarrhea and malabsorption in the second and third decades of life. 

Abdominal pain is usually a significant complaint, accompanied by weight loss and nutritional deficiencies. 

On physical examination, clubbing of the fingers may be noted and abdominal masses may be palpated.

IPSID is caused by a clonal proliferation of cells that produce an abnormal alpha heavy chain immunoglobulin.

 Diagnosed is by immunoassay for the alpha heavy chain in the serum. 

It is suggested that the clonal expansion is driven by an infectious antigen: similar to the link between H.pylori and mucosa-associated lymphoid tissue (MALT) lymphoma of the stomach. 

Campylobacter jejuni infection has been causally associated with IPSID.

Histological findings on mucosal biopsy of the small intestine reveals a dense cellular lymphoplasmacytic infiltrate in the lamina propria that results in crypt effacement.

The disease progresses from a relatively benign infiltration of the entire small intestinal mucosa to the development of lymphoplasmacytic and immunoblastic lymphoma.

IPSID is staged as other lymphomas.

Areas of bulky tumor are resected before chemotherapy and biopsy of enlarged mesenteric nodes is performed. 

In the premalignant stage A, long-term therapy with antibiotics such as tetracycline can cure the disease. 

In the more advanced stages of the disease (B and C), chemotherapy or total abdominal irradiation are used.

It rarely occurs at young ages.

Tobacco consumption and alcohol abuse contribute to the tumorigenesis. 

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