Primary gastrointestinal lymphomas involving the small bowel are uncommon accounting for less than 5% of all lymphomas. Conditions associated with small intestinal lymphomas include which of the following?

a. Acquired immune deficiency syndrome (AIDS)

b. Celiac disease

c. Crohn’s disease

d. Rheumatoid arthriti

Although rare, small intestinal lymphomas are associated with several conditions. The chronic malabsorptive condition, celiac disease, is recognized to be associated with small intestinal lymphoma. Clinical deterioration in a patient with previously controlled celiac disease should immediately suggest the diagnosis of lymphoma. There is also an increased incidence of lymphoma in Crohn’s disease. Disorders of immunologic function have an increased incidence of extranodal gastrointestinal lymphoma. These disorders include autoimmune diseases such as rheumatoid arthritis, Wegener granulomatosis, systemic lupus erythematous, and congenital immunodeficiencies. Immunosuppressed patients after organ transplantation and patients with prolonged, high-dose chemotherapy are also at increased risk. AIDS has been associated with the development of aggressive, non-Hodgkin’s lymphoma presenting with primary gastrointestinal involvement. Although this is usually a diffuse systemic disease, extranodal lymphoma of the small bowel has been frequently recognized.