Dear OncoLink "Ask the Experts,"
I would like specific information on: immunoproliferative small intestinal disease. I am a practicing Medical Oncologist, and would be grateful if I could get the latest on the disease.
Ken Blank, MD, and John Han-Chih Chang, MD, OncoLink Editorial Assistants, reply:Dear R.C.:
Lymphoma may involve the gastrointestinal tract either primarily or as a component of metastatic disease. Immunoproliferative small intestinal disease is one of five clinical types of primary small intestinal lymphoma. The other four are:
- adult Western,
- enteropathy-associated T cell lymphoma
- Hodgkin's disease.
More than half of patients with Mediterranean lymphoma present with an abdominal mass. A triad of signs/symptoms at presentation has been classically described and includes abdominal pain, nail clubbing and malabsorption. Immunoproliferative small intestinal disease usually involves the entire small intestine as the disease infiltrates and spreads throughout the mucosa of the small bowel. A diagnosis can usually be established by endoscopic biopsy of the second part of the small bowel (the jejunum). Chemotherapy is the mainstay of treatment. However, there are reports of successful utilization of whole abdominal radiotherapy. Surgery is usually reserved for symptoms such as obstruction, bleeding or perforation.