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IPSID

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IPSID,
IPSID
Immunoproliferative small intestinal disease Mediterranean lymphoma, α heavy chain disease A heterogeneous group of conditions characterized by monoclonal ↑ in production of Ig–usually α heavy chain, without accompanying light chains, ie 'truncated' immunoglobulins Clinical Malabsorption, diarrhea, weight loss, abdominal pain–due to marked expansion of the proximal small intestine and mesenteric lymphoid tissue, clubbing of fingers, toes Treatment Without antibiotic therapy–eg, tetracycline, IPSID may evolve to lymphoma or B cell immunoblastic sarcoma


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Patients with IPSID commonly have increased levels of a truncated immunoglobulin a heavy chain lacking an associated light chain, and thus the term a heavy chain disease (aHCD) is often used interchangeably with IPSID.
 
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