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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.


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Submitted January 30, 2004
Accepted June 30, 2004

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*

Department of Histopathology, Royal Free and University College Medical School, London, United Kingdom
Department of Pathology, University of Cambridge, Cambridge, United Kingdom
Institute of Pathology, Benjamin Franklin University Hospital, Free University, Berlin, Germany

* Corresponding author; email: p.isaacson{at}ucl.ac.uk.

MALT lymphomas usually arise at sites of acquired MALT, and are uncommon in native MALT (e.g. Peyer's 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 appendices) of marginal zone (MZ) hyperplasia in children aged 3-11 years, that, despite histological 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 intra-epithelial B-cells (IEBC). The MZ and IEBC showed a high proliferation fraction and a CD20+, CD21+, CD27-, IRTA-1+, CD43+, MUM-1, IgM+D+ phenotype. PCR, cloning and sequencing of rearranged IgH and Ig{lambda} genes (whole tissue sections [6 cases]; microdissected cells [2 cases]) showed that the MZ B-cells and IEBC were polyclonal and the IgH genes non-mutated. In contrast, marginal zone (intraepithelial) B-cells of 6 control tonsils had a similar immunophenotype, except for expression of CD27 and polytypic light chains while molecular studies showed that they were polyclonal with mutated Ig genes.


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