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The diagnosis is secured following biopsy of an involved nodal or extranodal site demonstrating a clonal B\u2010cell infiltrate with CD5 and CD10 negative immunophenotype most common. Some cases will features IgM paraprotein, but MYD88 L256P mutations are less frequent than in Waldenstrom macroglobulinemia. Prognostication Several prognostic models have been developed, including the MALT\u2010IPI and the MZL\u2010IPI. The latter is broadly applicable across MZL subtypes and incorporates elevated serum LDH, anemia, lymphopenia, thrombocytopenia and nodal or disseminated subtypes as independent predictors of outcome.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Treatment<\/jats:title>\n                    <jats:p>We discuss suggested approach to therapy for both early and advanced\u2010stage disease, with reference to chemo\u2010immunotherapy, radiotherapy, and emerging treatments in relapsed\/refractory disease such as BTK inhibitors.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ajh.27058","type":"journal-article","created":{"date-parts":[[2023,8,22]],"date-time":"2023-08-22T06:14:02Z","timestamp":1692684842000},"page":"1645-1657","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":67,"title":["Marginal zone lymphoma: 2023 update on diagnosis and management"],"prefix":"10.1002","volume":"98","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7988-1565","authenticated-orcid":false,"given":"Chan Y.","family":"Cheah","sequence":"first","affiliation":[{"name":"Department of Haematology Sir Charles Gairdner Hospital  Nedlands Australia"},{"name":"Medical School University of Western Australia  Crawley Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John F.","family":"Seymour","sequence":"additional","affiliation":[{"name":"Department of Haematology Peter MacCallum Cancer Centre and Royal Melbourne Hospital  Melbourne Australia"},{"name":"Sir Peter MacCallum Department of Oncology University of Melbourne  Parkville Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2023,8,21]]},"reference":[{"key":"e_1_2_6_2_1","first-page":"840","article-title":"Management of marginal zone lymphoma","volume":"27","author":"Reid R","year":"2013","journal-title":"Oncology (Williston Park)"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.beha.2016.07.002"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.21037\/aol-20-28"},{"key":"e_1_2_6_5_1","unstructured":"SwerdlowSH CampoE HarrisNL et al.WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. 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