title: Marginal zone lymphoma: Associated autoimmunity and auto-immune disorders.
abstract: Large epidemiological studies have shown a consistent increased risk for developing lymphoma in the setting of autoimmune disorders (AID). It is known that this link appears to be stronger for some AID and certain non-Hodgkin lymphoma subtypes e.g. Sjögren's syndrome and extra-nodal marginal zone lymphoma of the salivary gland, and thyroid MALT lymphoma in a background of Hashimoto's thyroiditis. B and T-cell hyperactivity due to chronic antigenic stimulation and the consequent presence of acquired lymphoid tissue seems to play a key role in the pathogenesis of AI-related lymphomas. Advanced age at diagnosis, prolonged disease course and disease severity are thought to increase the risk of lymphoma development in AID patients. There is increasing evidence that AI-related lymphomas constitute a different spectrum of entities indicating a different pathobiology with specific clinical features and treatment implications. This chapter will provide a general overview on the epidemiological aspects of the NHL-AID association focussing on marginal zone lymphomas - one of the NHL subtypes mostly implicated in the synchronous/metachronous association with AID. We will review the possible biological mechanisms involved and the risk factors in each autoimmune condition related to this lymphoma.
authors.affiliation: Histopathology Department, Royal Marsden Hospital, London, UK. Electronic address: Larissa.Sena@rmh.nhs.uk.
authors.forename: Larissa Sena
authors.initials: LS
authors.lastname: Teixeira Mendes
authors.affiliation: Histopathology Department, Royal Marsden Hospital, London, UK.
authors.forename: Andrew
authors.initials: A
authors.lastname: Wotherspoon
mesh_terms: D015551:Autoimmunity; D050031:Hashimoto Disease / Q000453:epidemiology / Q000276:immunology / Q000473:pathology; D006801:Humans; D018442:Lymphoma, B-Cell, Marginal Zone / Q000453:epidemiology / Q000276:immunology / Q000473:pathology; D012859:Sjogren's Syndrome / Q000453:epidemiology / Q000276:immunology / Q000473:pathology
keywords: Auto-immune diseases; Autoimmunity; Marginal zone lymphoma