Analysis of DNA from the mucosal tissue of three patients with immunoproliferative small intestinal disease (IPSID) and alpha chain disease, two of whom had early stage disease responsive to antibiotics, showed monoclonal heavy and light chain gene rearrangements in all cases. These findings suggest that IPSID is neoplastic even in its early stages, but that the neoplastic cells respond to normal stimuli. Monoclonal lymphoid populations could not be detected in circulating lymphocytes from these patients, which raises the possibility that the circulatory pathways of lymphocytes derived from human gut associated lymphoid tissue may not necessarily parallel those in experimental animals.
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