IPSID


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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

IPSID

immunoproliferative small intestinal disease.
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A histologic staging system for IPSID has been proposed, based on the hematoxylin-eosin appearance.
Gastrointestinal lymphomas: entities and mimics
Immunoproliferative small intestinal disease (IPSID): a model for mature B-cell neoplasms.
Classification of Gastrointestinal Lymphomasa B Cell Extranodal marginal zone, MALT type IPSID (heavy chain disease) Others (lymph node equivalents) Mantle cell (lymphomatous polyposis) Follicular Large B cell, NOS (~40% extranodal) Burkitt T Cell Enteropathy associated (EATL) Classic and type II Others (non-enteropathy associated) NK/T, nasal type [gamma][delta] ALCL Abbreviations: ALCL, anaplastic large cell lymphoma; EATL, enteropathy-associated T-cell lymphoma; IPSID, immunoproliferative small intestinal disease; MALT, mucosa-associated lymphoid tissue; NK, natural killer; NOS, not otherwise specified.
Lymphoproliferative disorders of the gastrointestinal tract: a review and pragmatic guide to diagnosis

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