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Pathogenetic Mechanisms Implicated in Sjogren's Syndrome Lymphomagenesis: A Review of the Literature.


ABSTRACT: Sjögren's Syndrome (SS) is a chronic autoimmune disorder characterized by focal mononuclear cell infiltrates that surround the ducts of the exocrine glands, impairing the function of their secretory units. Compared to other autoimmune disorders, SS is associated with a notably high incidence of non-Hodgkin lymphoma (NHL) and more frequently mucosa associated lymphoid tissue (MALT) lymphoma, leading to increased morbidity and mortality rates. High risk features of lymphoma development include systemic extraepithelial manifestations, low serum levels of complement component C4 and mixed type II cryoglobulinemia. The discrimination between reactive and neoplastic lymphoepithelial lesion (LEL) is challenging, probably reflecting a continuum in the evolution from purely inflammatory lymphoid in

SUBMITTER: Stergiou IE 

PROVIDER: S-EPMC7759999 | biostudies-literature | 2020 Nov

REPOSITORIES: biostudies-literature