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Blood, 15 March 2005, Vol. 105, No. 6, pp. 2274-2280. Prepublished online as a Blood First Edition Paper on November 12, 2004; DOI 10.1182/blood-2004-07-2755. This Article Full Text (PDF) All Versions of this Article: 2004-07-2755v1 105/6/2274    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Al-Saleem, T. Articles by Al-Mondhiry, H. Search for Related Content PubMed PubMed Citation Articles by Al-Saleem, T. Articles by Al-Mondhiry, H. Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted July 23, 2004 Accepted October 28, 2004 Immunoproliferative small intestinal disease (IPSID); a model for mature B-cell neoplasms Tahseen Al-Saleem* and Hamid Al-Mondhiry Department of Pathology, Fox Chase Cancer Center, Philadelphia, PA, USA Division of Hematology/Oncology, Milton S. Hershey Medical Center and the Pennsylvania State University College of Medicine, Hershey, PA, USA * Corresponding author; email: T_AlSaleem{at}fccc.edu. 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 heavy chain proteins lacking the light chains as well as the first constant domain. The corresponding mRNA lacks the VH and the CH1 sequences and contains deletions as well as insertions of unknown origin. The encoding gene sequence reveals a deletion of V region and parts of CH1 domain. Cytogenetic studies demonstrated clonal rearrangements involving predominantly the heavy and light chain genes, including t9;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, mesenteric lymph nodes, and may metastasize to a distant organ. IPSID lymphoma shares clinical, morphological and molecular features with MALT lymphoma, lymphoplasmacytic lymphoma and with plasma cell neoplasms. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: N. C. Munshi, S. Digumarthy, and A. Rahemtullah Case 13-2008 -- A 46-Year-Old Man with Rheumatoid Arthritis and Lymphadenopathy N. Engl. J. Med., April 24, 2008; 358(17): 1838 - 1848. [Full Text] [PDF] C. S. Portlock, P. Hamlin, A. Noy, W. Chey, C. A. Gaydos, L. Palomba, I. Schwartz, S. Corcoran, L. Rosenzweig, D. Walker, et al. Infectious disease associations in advanced stage, indolent lymphoma (follicular and nonfollicular): developing a lymphoma prevention strategy Ann. Onc., February 1, 2008; 19(2): 254 - 258. [Abstract] [Full Text] [PDF] E. Zucca and F. Bertoni Another Piece of the MALT Lymphomas Jigsaw J. Clin. Oncol., August 1, 2005; 23(22): 4832 - 4834. [Full Text] [PDF]

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