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Primary lymphoma of the small intestine may be one of two types. The Western type is a focal tumour of lymphoid tissue, 20% of which are multiple. The Mediterranean type is a form of immunoproliferative disease characterized by a proliferation of lymphoid tissue along the whole length of the small intestine. It is associated with alpha-chain disease, in which there is a proliferation of plasma cells in the lamina propria of the small intestine, with a pre-malignant potential.
The small intestine may be involved in secondary lymphoma, but a primary form is more likely in the absence of lymphomatosis, lymphadenopathy or hepatosplenomegaly.
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