Same Disease, Different Approaches: A Report on Six Lymphoma Cases with Extranodal Marginal Zones in Rare Sites

Abstract

34 Marginal zone lymphomas (MZL) constitute around 10% of non-Hodgkins lymphomas. Extranodal marginal zone lymphoma (EMZL) is the most common form of MZL, constituting more than 70% of all MZL cases.1 Marginal zone lymphoma can be considered as a prototype of an antigen-driven tumor and has many microorganisms and autoimmune diseases associated with its pathogenesis.2 A pooled analysis of a series of trials showed that Sjögren’s Syndrome could increase the risk of MZL and EMZL of the parotid gland to 30 and 1000-folds, respectively. A similar association was also reported with systemic lupus erythematosus.3 The main causative infectious agents of the EMZL that were also associated with the specific disease locations were Helicobacter pylori, Chlamydia psittaci, Campylobacter jejuni, Borrelia burgdorferi, and Achromobacter xylosoxidans.4