{"entity":{"id":"nodal-marginal-zone-lymphoma","kind":"cancer","name":"Nodal marginal zone lymphoma","aka":["NMZL","Nodal marginal zone B cell lymphoma","Nodal marginal zone B-cell lymphoma","Monocytoid B-cell lymphoma","Paediatric nodal marginal zone lymphoma"],"tldr":"Nodal marginal zone lymphoma is a slow-growing lymphoma of the lymph nodes that looks like the MALT and splenic types under the microscope but has no organ or spleen involvement to explain it. It lacks a diagnostic marker, so it is diagnosed by excluding the other small B-cell lymphomas, and it is treated like follicular lymphoma with rituximab-based therapy.","summary":"WHO-HAEM5 keeps nodal marginal zone lymphoma as the nodal member of the family, distinguished from splenic marginal zone lymphoma by its pattern of dissemination and still lacking distinct markers (Alaggio 2022; Spina 2016). Exome, targeted and transcriptome sequencing of 35 cases found a distinctive pattern of lesions: KMT2D (MLL2) mutated in 34 percent, PTPRD in 20 percent, NOTCH2 in 20 percent and KLF2 in 17 percent, with PTPRD mutations enriched in this lymphoma among mature B-cell tumours and linked to loss of phosphatase activity and increased proliferation (Spina 2016). Gene expression profiling against follicular lymphoma identified enriched interleukin, integrin, CD40, PI3K and NF-kB pathways, high SYK and TACI expression, and higher CHIT1, TGFB1 and TACI than the BCL6, LMO2 and CD10 of follicular lymphoma (Blood 2012). A paediatric type with an indolent course is recognised.\n\nHow it differs from its parent: no infection to eradicate and no spleen to remove; it presents like other indolent nodal lymphomas and its main difficulty is telling it from follicular lymphoma and from nodal spread of an extranodal or splenic marginal zone lymphoma.\n\nHow common: no registry share in the sources read; rare.\n\nTreatment: as for follicular lymphoma of the same stage, following the ESMO marginal zone guideline: involved-site radiotherapy for localised disease, watch and wait or rituximab with or without chemotherapy for advanced disease, and lenalidomide-rituximab or BTK inhibitors on relapse (Zucca 2020).","asOf":"2026-09-24","wikipedia":"https://en.wikipedia.org/wiki/Marginal_zone_B-cell_lymphoma","links":[{"label":"NCI PDQ: adult non-Hodgkin lymphoma treatment","url":"https://www.cancer.gov/types/lymphoma/patient/adult-nhl-treatment-pdq"},{"label":"Alaggio 2022, Leukemia: the 5th edition WHO classification of haematolymphoid tumours, lymphoid neoplasms","url":"https://doi.org/10.1038/s41375-022-01620-2"},{"label":"Zucca 2020, Annals of Oncology: marginal zone lymphomas, ESMO clinical practice guidelines","url":"https://doi.org/10.1016/j.annonc.2019.10.010"},{"label":"Spina 2016, Blood: the genetics of nodal marginal zone lymphoma","url":"https://doi.org/10.1182/blood-2016-02-696757"},{"label":"Blood 2012: nodal marginal zone lymphoma gene expression and miRNA profiling","url":"https://doi.org/10.1182/blood-2011-02-339556"}],"tags":["subtype-page","wave4","haematologic","rare"],"related":["marginal-zone-lymphoma","malt-lymphoma","splenic-marginal-zone-lymphoma","follicular-lymphoma"],"cancers":[],"sections":[],"technologies":[],"targets":[],"drugs":["rituximab","bendamustine","lenalidomide","zanubrutinib"],"companies":[],"institutions":[],"pathways":[],"terms":[],"trials":["augment","fort"],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"group":"haematologic","burden":"Rare; the largest genetic study holds 35 patients (Spina 2016). The ESMO guideline gives it as the rarest or second rarest of the three marginal zone lymphomas; no registry share is in the sources read.","subtypes":["Nodal marginal zone lymphoma, adult type (KMT2D, PTPRD, NOTCH2 and KLF2 mutations)","Paediatric nodal marginal zone lymphoma (indolent, localised, young males)","Nodal marginal zone lymphoma with plasmacytic differentiation"],"biomarkers":["Exclusion immunophenotype: CD20 positive, CD5, CD10, cyclin D1 and BCL6 negative","KMT2D, PTPRD, NOTCH2 and KLF2 mutations","Absence of extranodal or splenic disease on staging"],"standardOfCare":[{"setting":"All stages","approach":"Treated as follicular lymphoma of the same stage: radiotherapy when localised, rituximab with or without chemotherapy when advanced, lenalidomide-rituximab or BTK inhibitors on relapse.","refs":["follicular-lymphoma","rituximab","bendamustine","lenalidomide","zanubrutinib"]}],"stateOfArt":[],"history":[],"pipeline":[],"openProblems":[],"parent":"marginal-zone-lymphoma"},"route":"/cancers/nodal-marginal-zone-lymphoma/","neighbours":{"cancer":[{"id":"malt-lymphoma","kind":"cancer","name":"Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma)","route":"/cancers/malt-lymphoma/"},{"id":"follicular-lymphoma","kind":"cancer","name":"Follicular lymphoma","route":"/cancers/follicular-lymphoma/"},{"id":"marginal-zone-lymphoma","kind":"cancer","name":"Marginal zone lymphoma","route":"/cancers/marginal-zone-lymphoma/"},{"id":"splenic-marginal-zone-lymphoma","kind":"cancer","name":"Splenic marginal zone lymphoma","route":"/cancers/splenic-marginal-zone-lymphoma/"}],"drug":[{"id":"bendamustine","kind":"drug","name":"Bendamustine","route":"/drugs/bendamustine/"},{"id":"lenalidomide","kind":"drug","name":"Lenalidomide","route":"/drugs/lenalidomide/"},{"id":"rituximab","kind":"drug","name":"Rituximab","route":"/drugs/rituximab/"},{"id":"zanubrutinib","kind":"drug","name":"Zanubrutinib","route":"/drugs/zanubrutinib/"}],"trial":[{"id":"augment","kind":"trial","name":"AUGMENT","route":"/trials/augment/"},{"id":"fort","kind":"trial","name":"FoRT","route":"/trials/fort/"}]}}