title: Mucosa-associated lymphoid tissue lymphoma in Sjögren's syndrome: risks, management, and prognosis.
abstract: Sjögren's syndrome is a chronic inflammatory disease primarily affecting the exocrine glands. Its association with lymphoma is well documented, with salivary extranodal marginal zone lymphomas of the mucosa-associated lymphoid tissue type being the most common and constituting a major disease complication. These neoplasms are antigen-stimulated B-cell lymphomas characterized by localized stage, indolent clinical course, and recurrence in other extranodal sites. This article presents a review of the literature and discusses the clinical, histopathologic, therapeutic, and prognostic aspects of these tumors in Sjögren's syndrome. In addition, it highlights the predictor markers of lymphoma development.
authors.affiliation: Department of Pathophysiology, Medical School, National University of Athens, 75 Mikras Asias Street, 11527 Athens, Greece. mvoulgar@med.uoa.gr
authors.forename: Michael
authors.initials: M
authors.lastname: Voulgarelis
authors.forename: Haralampos M
authors.initials: HM
authors.lastname: Moutsopoulos
mesh_terms: D015551:Autoimmunity; D006801:Humans; D018442:Lymphoma, B-Cell, Marginal Zone / Q000209:etiology* / Q000473:pathology / Q000628:therapy; D015201:Meta-Analysis as Topic; D011379:Prognosis; D012307:Risk Factors; D012859:Sjogren's Syndrome / Q000150:complications* / Q000473:pathology / Q000628:therapy