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, Peter G. Isaacson

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69 Scopus citations

Abstract

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 Igλ 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.

Original languageEnglish (US)
Pages (from-to)3343-3348
Number of pages6
JournalBlood
Volume104
Issue number10
DOIs
StatePublished - Nov 15 2004

ASJC Scopus subject areas

  • Biochemistry
  • Immunology
  • Hematology
  • Cell Biology

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    Attygalle, A. D., Liu, H., Shirali, S., Diss, T. C., Loddenkemper, C., Stein, H., Dogan, A., Du, M. Q., & Isaacson, P. G. (2004). Atypical marginal zone hyperplasia of mucosa-associated lymphoid tissue: A reactive condition of childhood showing immunoglobulin lambda light-chain restriction. Blood, 104(10), 3343-3348. https://doi.org/10.1182/blood-2004-01-0385