Extranodal marginal zone lymphoma, splenic marginal zone lymphoma, and nodal marginal zone lymphoma.
Marginal zone lymphomas, also known as marginal zone B-cell lymphomas (MZLs), are a heterogeneous group of lymphomas that derive from the malignant transformation of marginal zone B-cells.[1] Marginal zone B cells are innate lymphoid cells that normally function by rapidly mounting IgM antibody immune responses to antigens such as those presented by infectious agents and damaged tissues.[2] They are lymphocytes of the B-cell line that originate and mature in secondary lymphoid follicles and then move to the marginal zones of mucosa-associated lymphoid tissue (MALT), the spleen, or lymph nodes. Mucosa-associated lymphoid tissue is a diffuse system of small concentrations of lymphoid tissue found in various submucosal membrane sites of the body such as the gastrointestinal tract, mouth, nasal cavity, pharynx, thyroid gland, breast, lung, salivary glands, eye, skin and the human spleen.[3]
In 2016, the World Health Organization classified MZLs into three different types. Extranodal marginal zone lymphomas (EMZLs) are MZLs that develop in extranodal tissues. Most EMZLs develop in MALT and are often termed extranodal MZL of mucosa-associated lymphoid tissue or, more simply, MALT lymphomas. Splenic marginal zone lymphomas (SMZLs) are MZLs that initially are confined to the spleen, bone marrow, and blood.[1]Nodal marginal zone lymphomas (NMZs) are MZLs initially confined to lymph nodes, bone marrow, and blood.[1] While all of these MZL involve malignant B-cells, they differ not only in the tissues they involve but also in their pathophysiology, clinical presentations, prognoses, and treatments.[1][4]
MZLs represent 5–17% of all Non-Hodgkin lymphomas with the extranodal, splenic, and nodal forms accounting for 50–70%, ~20%, and ~10% of all MZLs.[5] The three MZL subtypes occur more often in older people (age 65–68 years) and are indolent diseases that may, in people without symptoms, be initially treated by a watchful waiting strategy. However, NMZL carries a somewhat worse long term outcome than the other subtypes[1] and any of the MZL subtypes may progress in a low percentage of cases to a more aggressive lymphoma, particularly diffuse large B-cell lymphoma.[6] One of the most distinctive feature of MZL is that many cases are associated with the persistent simulation of the immune system by the chronic inflammation that accompanies infections[7] or autoimmune diseases.[8] MZL cases associated with certain infectious pathogens can be cured by treatment directed at the pathogens causing or associated with these infections.[7]
^ abcdeBron D, Meuleman N (September 2019). "Marginal zone lymphomas: second most common lymphomas in older patients". Current Opinion in Oncology. 31 (5): 386–393. doi:10.1097/CCO.0000000000000554. PMID 31246587. S2CID 195765608.
^Thieblemont C, Zucca E (2017). "Clinical aspects and therapy of gastrointestinal MALT lymphoma". Best Practice & Research. Clinical Haematology. 30 (1–2): 109–117. doi:10.1016/j.beha.2017.01.002. PMID 28288705.
^Sriskandarajah P, Dearden CE (2017). "Epidemiology and environmental aspects of marginal zone lymphomas". Best Practice & Research. Clinical Haematology. 30 (1–2): 84–91. doi:10.1016/j.beha.2016.07.002. PMID 28288721.
^Swerdlow SH, Campo E, Pileri SA, Harris NL, Stein H, Siebert R, Advani R, Ghielmini M, Salles GA, Zelenetz AD, Jaffe ES (May 2016). "The 2016 revision of the World Health Organization classification of lymphoid neoplasms". Blood. 127 (20): 2375–90. doi:10.1182/blood-2016-01-643569. PMC 4874220. PMID 26980727.
^Cheah CY, Opat S, Trotman J, Marlton P (February 2019). "Front-line management of indolent non-Hodgkin lymphoma in Australia. Part 2: mantle cell lymphoma and marginal zone lymphoma" (PDF). Internal Medicine Journal. 49 (9): 1070–1080. doi:10.1111/imj.14268. PMID 30816618. S2CID 73491365.
^Casulo C, Friedberg J (2017). "Transformation of marginal zone lymphoma (and association with other lymphomas)". Best Practice & Research. Clinical Haematology. 30 (1–2): 131–138. doi:10.1016/j.beha.2016.08.029. PMID 28288708.
^ abSmedby KE, Ponzoni M (November 2017). "The aetiology of B-cell lymphoid malignancies with a focus on chronic inflammation and infections". Journal of Internal Medicine. 282 (5): 360–370. doi:10.1111/joim.12684. PMID 28875507.
^Nocturne G, Pontarini E, Bombardieri M, Mariette X (March 2019). "Lymphomas complicating primary Sjögren's syndrome: from autoimmunity to lymphoma". Rheumatology. 60 (8): 3513–3521. doi:10.1093/rheumatology/kez052. PMC 8328496. PMID 30838413.
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