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Blood, 15 November 2004, Vol. 104, No. 10, pp. 3343-3348. Prepublished online as a Blood First Edition Paper on July 15, 2004; DOI 10.1182/blood-2004-01-0385. This Article Full Text Full Text (PDF) All Versions of this Article: 2004-01-0385v1 104/10/3343    most recent Alert me when this article is cited Alert me if a correction is posted Citation Map 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 Attygalle, A. D. Articles by Isaacson, P. G. Search for Related Content PubMed PubMed Citation Articles by Attygalle, A. D. Articles by Isaacson, P. G. Related Collections Immunobiology Neoplasia Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  NEOPLASIA Atypical marginal zone hyperplasia of mucosa-associated lymphoid tissue: a reactive condition of childhood showing immunoglobulin lambda light-chain restriction Ayoma D. Attygalle, Hongxiang Liu, Sima Shirali, Timothy C. Diss, Christoph Loddenkemper, Harald Stein, Ahmet Dogan, Ming-Qing Du, and Peter G. Isaacson From the Department of Histopathology, Royal Free and University College Medical School, London, United Kingdom; Department of Pathology, University of Cambridge, Cambridge, United Kingdom; and Institute of Pathology, Benjamin Franklin University Hospital, Free University Berlin, Berlin, Germany. Mucosa-associated lymphoid tissue (MALT) lymphomas usually arise at sites of acquired MALT and are uncommon in native MALT (eg, Peyer patches and tonsil). Malignancy in these low-grade lymphomas is often inferred by immunoglobulin light-chain restriction and expression of CD43; molecular genetic evidence is sought only if these are in doubt. We report 6 cases (4 tonsils, 2 appendixes) of marginal zone (MZ) hyperplasia in children aged 3 to 11 years that, despite histologic and immunophenotypic features indicative of lymphoma, were polyclonal by molecular analysis. No lymphoma-directed therapy was given and patients remain alive and well (5 cases, median follow-up 35.3 months). The involved tonsil and appendix showed florid MZ hyperplasia with prominent intraepithelial B cells (IEBCs). The MZ B cells and IEBCs showed a high-proliferation fraction and a CD20+, CD21+, CD27-, immunoglobulin (Ig) superfamily receptor translocation-associated 1-positive (IRTA-1+), CD43+, multiple myeloma oncogene 1 (MUM-1), IgM+D+ phenotype. Polymerase chain reaction (PCR), cloning, and sequencing of rearranged IgH and Ig genes (whole tissue sections [6 cases]; microdissected cells [2 cases]) showed that the MZ B cells and IEBCs were polyclonal and the IgH genes nonmutated. In contrast, MZ (intraepithelial) B cells of 6 control tonsils had a similar immunophenotype, except for expression of CD27 and polytypic light chains, whereas molecular studies showed that they were polyclonal with mutated Ig genes. (Blood. 2004;104:3343-3348) CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: M. Onciu The Pathologist's Perspective on Challenging Pediatric Lymphomas: Update on the Pathobiology of Uncommon Childhood Lymphomas ASCO Educational Book, January 1, 2009; 2009(1): 604 - 607. [Abstract] [Full Text] [PDF] F. E. Craig and K. A. Foon Flow cytometric immunophenotyping for hematologic neoplasms Blood, April 15, 2008; 111(8): 3941 - 3967. [Abstract] [Full Text] [PDF] G. R.A. Ehrhardt, J. T. Hsu, L. Gartland, C.-M. Leu, S. Zhang, R. S. Davis, and M. D. Cooper Expression of the immunoregulatory molecule FcRH4 defines a distinctive tissue-based population of memory B cells J. Exp. Med., September 19, 2005; 202(6): 783 - 791. [Abstract] [Full Text] [PDF]

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