Immunoproliferative small intestinal disease (IPSID): a model for mature B-cell neoplasms

Blood. 2005 Mar 15;105(6):2274-80. doi: 10.1182/blood-2004-07-2755. Epub 2004 Nov 12.

Abstract

Immunoproliferative small intestinal disease (IPSID) was recently added to the growing list of infectious pathogen-associated human lymphomas. Molecular and immunohistochemical studies demonstrated an association with Campylobacter jejuni. IPSID is a variant of the B-cell lymphoma of mucosa-associated lymphoid tissue (MALT), which involves mainly the proximal small intestine resulting in malabsorption, diarrhea, and abdominal pain. Geographically, IPSID is most prevalent in the Middle East and Africa. IPSID lymphomas reveal excessive plasma cell differentiation and produce truncated alpha heavy chain proteins lacking the light chains as well as the first constant domain. The corresponding mRNA lacks the variable heavy chain (V(H)) and the constant heavy chain 1 (C(H)1) sequences and contains deletions as well as insertions of unknown origin. The encoding gene sequence reveals a deletion of V region and parts of C(H)1 domain. Cytogenetic studies demonstrated clonal rearrangements involving predominantly the heavy and light chain genes, including t(9;14) translocation involving the PAX5 gene. Early-stage IPSID responds to antibiotics (30%-70% complete remission). Most untreated IPSID patients progress to lymphoplasmacytic and immunoblastic lymphoma invading the intestinal wall and mesenteric lymph nodes, and may metastasize to a distant organ. IPSID lymphoma shares clinical, morphologic, and molecular features with MALT lymphoma, lymphoplasmacytic lymphoma, and plasma cell neoplasms.

Publication types

  • Review

MeSH terms

  • Adolescent
  • Adult
  • Africa
  • Campylobacter Infections* / complications
  • Campylobacter Infections* / genetics
  • Campylobacter Infections* / immunology
  • Campylobacter Infections* / pathology
  • Campylobacter Infections* / therapy
  • Campylobacter jejuni*
  • Child
  • Chromosomes, Human, Pair 14
  • Chromosomes, Human, Pair 9 / genetics
  • Chromosomes, Human, Pair 9 / immunology
  • Female
  • Humans
  • Immunoglobulin Light Chains / genetics
  • Immunoglobulin Light Chains / immunology
  • Immunoglobulin Variable Region / genetics
  • Immunoglobulin Variable Region / immunology
  • Immunoglobulin alpha-Chains / genetics
  • Immunoglobulin alpha-Chains / immunology
  • Immunoproliferative Small Intestinal Disease* / etiology
  • Immunoproliferative Small Intestinal Disease* / genetics
  • Immunoproliferative Small Intestinal Disease* / immunology
  • Immunoproliferative Small Intestinal Disease* / pathology
  • Immunoproliferative Small Intestinal Disease* / therapy
  • Intestine, Small / immunology
  • Intestine, Small / pathology
  • Lymph Nodes / immunology
  • Lymph Nodes / pathology
  • Lymphoma, B-Cell, Marginal Zone* / etiology
  • Lymphoma, B-Cell, Marginal Zone* / immunology
  • Lymphoma, B-Cell, Marginal Zone* / pathology
  • Lymphoma, B-Cell, Marginal Zone* / therapy
  • Male
  • Mesentery / immunology
  • Mesentery / pathology
  • Middle East
  • PAX5 Transcription Factor / genetics
  • PAX5 Transcription Factor / immunology
  • Plasma Cells / immunology*
  • Plasma Cells / pathology
  • Sequence Deletion / genetics
  • Sequence Deletion / immunology
  • Translocation, Genetic / genetics
  • Translocation, Genetic / immunology

Substances

  • Immunoglobulin Light Chains
  • Immunoglobulin Variable Region
  • Immunoglobulin alpha-Chains
  • PAX5 Transcription Factor
  • PAX5 protein, human