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Blood, 15 October 2008, Vol. 112, No. 8, pp. 3355-3361.
Prepublished online as a Blood First Edition Paper on August 7, 2008; DOI 10.1182/blood-2008-01-132415.


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Submitted January 18, 2008
Accepted July 18, 2008

The majority of cutaneous marginal zone B cell lymphomas express class switched immunoglobulins and develop in a T helper type 2 inflammatory environment

Febe van Maldegem, Remco van Dijk, Thera A.M. Wormhoudt, Philip M. Kluin, Rein Willemze, Lorenzo Cerroni, Carel J. M. van Noesel*, and Richard J. Bende

Department of Pathology, Academic Medical Center, Amsterdam, Netherlands
Department of Pathology and Laboratory Medicine, University Medical Center Groningen, Groningen, Netherlands
Department of Dermatology, Leiden University Medical Center, Leiden, Netherlands
Department of Dermatology, Medical University of Graz, Graz, Austria

* Corresponding author; email: c.j.vannoesel{at}amc.uva.nl.

Extranodal marginal zone B cell lymphomas (MZBCLs) arise on a background of chronic inflammation due to organ-specific autoimmunity, infection, or by unknown causes. Well known examples are salivary gland MZBCL in Sjogren's sialadenitis and gastric MZBCL in Helicobacter pylori gastritis. MZBCLs express CXCR3, a receptor for IFN-{gamma}-induced chemokines highly expressed in the chronic inflammatory environment. The immunoglobulin variable heavy/light chain (IgVH/IgVL) gene repertoire of salivary gland- and gastric- MZBCL appears restricted and frequently encodes BCRs with rheumatoid factor (RF) reactivity. Primary cutaneous marginal zone B cell lymphomas (PCMZLs) are regarded as the skin-involving counterpart of extranodal MZBCL. Although PCMZLs have been associated with Borrelia burgdorferi dermatitis, PCMZLs generally arise due to unknown causes. We studied an extensive panel of PCMZLs and show that PCMZLs do not conform to the general profile of extranodal MZBCL. Whereas most non-cutaneous MZBCLs express IgM, PCMZL in majority express IgG, IgA and IgE, and do not show an obvious immunoglobulin repertoire bias. Furthermore, the isotype-switched PCMZLs lack CXCR3 and seem to arise in a different inflammatory environment, as compared to other extranodal MZBCL.


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