Lymphoid hyperplasia
Focal hyperplasia occurs in the terminal ileum of young children where it may cause intussusception. In older adults, it may form multiple polyps in the rectosigmoid.
Diffuse hyperplasia may occur innocuously without immunodeficiency. When associated with immunodeficiency there is some risk of malignant transformation. The most common association is with variable immunodeficiency with hypogammaglobulinaemia and a risk of parasitic infections such as giardiasis.
Most cases occur in the stomach in association with Helicobacter infection. Follicular colonisation may mimic follicular lymphoma.
Occurs in relatively young females. Most common in the duodenum. The prognosis of restricted disease is good. Differential diagnosis:
Lymphomatoid polyposis (mantle cell lymphoma).
Extranodal marginal zone lymphoma with follicular colonisation
Some arise by transformation of low-grade marginal zone lymphoma or mantle cell lymphoma. Staging, specifically involvement of regional lymph nodes, is prognostically important. Some cases arise by transformation of extranodal marginal zone lymphoma.
Burkitt lymphoma usually presents with obstruction of the terminal ileum by tumour in a young patient.
Mantle cell lymphoma: commonly forms lymphomatoid polyposis.
Immunoproliferative small intestinal disease: this entity was first described in the Mediterranean and Middle East, where it is thought to be associated with chronic enteric infections, particularly Campylobacter. The lamina propria shows plasmacytosis with eosinophilia. Even when it reaches the stage of forming a tumour mass, the infiltrate remains polymorphous. There may be production of abnormal incomplete immunoglobulin a heavy chain.
Plasmacytoma: demonstration of light chain restriction is useful in the differentiation from plasmacytic hyperplasia. The differentiation from extranodal marginal zone lymphoma with plasmacytic differentiation may be difficult.
Peripheral T-cell lymphoma of no special type is not associated with coeliac disease. The neoplastic T-cells may be obscured by a mixed inflammatory component.
Enteropathy-type T-cell lymphoma Refractory sprue and ulcerative jejunitis are thought to represent the earliest stage of the development of an lymphomatous progression in coeliac disease. Epitheliotropic T-cell lymphoma is a variant.
Hodgkin lymphoma is rare in the gastrointestinal tract, usually occurring in the elderly and in association with immunodeficiency.
References
1 Banks PM. Gastrointestinal lymphoproliferative disorders. Histopathology 2007; 50:42-54
This page last revised 15.2.2007.
©SMUHT/PW Bishop