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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.
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.
How 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.
How common: no registry share in the sources read; rare.
Treatment: 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).
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.
Leukaemias, myeloma and MDS live in the marrow and blood; lymphomas grow in lymph nodes and spleen. The node stations are the disease map, not a route of spread, and staging counts them.
In lymphoma the node stations are the disease itself; staging (Ann Arbor / Lugano) counts how many regions and sides of the diaphragm are involved.
Same organ: High-grade B-cell lymphoma with MYC and BCL2 rearrangements (double-hit lymphoma), Mediastinal grey zone lymphoma, Primary effusion lymphoma, Plasmablastic lymphoma, T-cell/histiocyte-rich large B-cell lymphoma, EBV-positive diffuse large B-cell lymphoma, Primary large B-cell lymphoma of the testis, Gastric MALT lymphoma, Ocular adnexal MALT lymphoma, Extranodal NK/T-cell lymphoma, Adult T-cell leukaemia/lymphoma, ALK-positive anaplastic large cell lymphoma, ALK-negative anaplastic large cell lymphoma, Breast implant-associated anaplastic large cell lymphoma, Primary cutaneous anaplastic large cell lymphoma, Lymphomatoid papulosis, Mycosis fungoides, Enteropathy-associated T-cell lymphoma, Monomorphic epitheliotropic intestinal T-cell lymphoma, Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma), Splenic marginal zone lymphoma, Primary cutaneous marginal zone lymphoma, Primary cutaneous follicle centre lymphoma, Sezary syndrome, Nodal T-follicular helper cell lymphoma, angioimmunoblastic type (angioimmunoblastic T-cell lymphoma), Hepatosplenic T-cell lymphoma, Intravascular large B-cell lymphoma, Lymphomatoid granulomatosis, T-cell prolymphocytic leukaemia, Splenic B-cell lymphoma/leukaemia with prominent nucleoli (formerly B-cell prolymphocytic leukaemia and hairy cell leukaemia variant), T-cell large granular lymphocytic leukaemia, Mixed-phenotype acute leukaemia, Myeloid leukaemia of Down syndrome, Burkitt leukaemia, Marginal zone lymphoma, Cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome), Primary mediastinal (thymic) large B-cell lymphoma, Leukaemia (all types), Acute myeloid leukaemia, Acute lymphoblastic leukaemia, Chronic lymphocytic leukaemia, Chronic myeloid leukaemia (CML), Diffuse large B-cell lymphoma, Follicular lymphoma, Hodgkin lymphoma, Mantle cell lymphoma, Multiple myeloma, Non-Hodgkin lymphoma (all types), Myelodysplastic syndromes / neoplasms (MDS), Myeloproliferative neoplasms (PV, ET, myelofibrosis), Polycythaemia vera (PV), Essential thrombocythaemia (ET), Waldenström macroglobulinaemia, Hairy cell leukaemia, Peripheral T-cell lymphomas (including cutaneous T-cell lymphoma), Blastic plasmacytoid dendritic cell neoplasm (BPDCN), Burkitt lymphoma, HIV-associated (AIDS-related) lymphomas, Chronic myelomonocytic leukaemia and MDS/MPN overlap neoplasms, Systemic mastocytosis, Erdheim-Chester disease, Rosai-Dorfman disease and other histiocytic neoplasms, Langerhans cell histiocytosis (LCH), Post-transplant lymphoproliferative disorder (PTLD), FLT3-mutated acute myeloid leukaemia, IDH1- and IDH2-mutated acute myeloid leukaemia, NPM1-mutated and KMT2A-rearranged acute myeloid leukaemia, Secondary and therapy-related acute myeloid leukaemia, Acute promyelocytic leukaemia, Acute myeloid leukaemia in older or unfit patients, Smouldering multiple myeloma, Newly diagnosed multiple myeloma, transplant-eligible, Newly diagnosed multiple myeloma, transplant-ineligible, Relapsed or refractory multiple myeloma, Plasma cell leukaemia, Lower-risk myelodysplastic syndromes, Higher-risk myelodysplastic syndromes, Chronic lymphocytic leukaemia, first treatment, Relapsed or refractory chronic lymphocytic leukaemia, Richter transformation of chronic lymphocytic leukaemia, Chronic myeloid leukaemia, chronic phase, Chronic myeloid leukaemia, accelerated and blast phase, Primary myelofibrosis, Standard-risk B-cell acute lymphoblastic leukaemia in children, High-risk acute lymphoblastic leukaemia in children (high-risk B-ALL and T-ALL), Philadelphia chromosome-positive acute lymphoblastic leukaemia in children (Ph-positive ALL), Philadelphia chromosome-like acute lymphoblastic leukaemia (Ph-like or BCR::ABL1-like ALL), Infant acute lymphoblastic leukaemia (KMT2A-rearranged, under one year), Relapsed and refractory acute lymphoblastic leukaemia in children, Acute myeloid leukaemia in children, Erdheim-Chester disease, Rosai-Dorfman-Destombes disease, Single-system Langerhans cell histiocytosis (bone, skin or one other organ), Multisystem Langerhans cell histiocytosis (with or without risk-organ involvement), Indolent and smouldering systemic mastocytosis, Advanced systemic mastocytosis (aggressive SM, SM with an associated haematological neoplasm, mast cell leukaemia), Early-stage classical Hodgkin lymphoma (stage I to II), Advanced-stage classical Hodgkin lymphoma (stage III to IV), Nodular lymphocyte-predominant Hodgkin lymphoma (nodular lymphocyte-predominant B-cell lymphoma), Relapsed and refractory classical Hodgkin lymphoma
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Also on OnCo: Symptoms and red flags · Early detection roadmap.
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.
Nodal marginal zone lymphoma has no site-specific treatment of its own and is managed on the follicular lymphoma pathway: watch and wait while asymptomatic, involved-site radiotherapy at 24 Gy for genuinely localised disease, and rituximab-based chemoimmunotherapy (bendamustine and rituximab, or R-CVP) when the disease causes symptoms, organ compromise or cytopenias. Lenalidomide with rituximab is supported by AUGMENT, which enrolled marginal zone as well as follicular lymphoma and gave median progression-free survival of 39.4 against 14.1 months for rituximab alone. Zanubrutinib is an option at relapse on the strength of MAGNOLIA. Hepatitis C should be tested for, as in the splenic form. Transformation to diffuse large B-cell lymphoma is treated as aggressive lymphoma.
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Query for this cancer: (TITLE:"Nodal marginal zone lymphoma" OR ABSTRACT:"Nodal marginal zone lymphoma" OR TITLE:"NMZL" OR ABSTRACT:"NMZL" OR TITLE:"Nodal marginal zone B cell lymphoma" OR ABSTRACT:"Nodal marginal zone B cell lymphoma" OR TITLE:"Nodal marginal zone B-cell lymphoma" OR ABSTRACT:"Nodal marginal zone B-cell lymphoma" OR TITLE:"Monocytoid B-cell lymphoma" OR ABSTRACT:"Monocytoid B-cell lymphoma" OR TITLE:"Paediatric nodal marginal zone lymphoma" OR ABSTRACT:"Paediatric nodal marginal zone lymphoma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Nodal marginal zone lymphoma, not a curated reading list.
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Breathing very fast, confusion or slurred speech, blue, pale or blotchy skin, a very high or very low temperature, shivering, or a rash that does not fade when pressed: the NHS says call 999 or go to A and E, and do not drive yourself.
Bleeding that does not stop by itself, bleeding from more than one site, or new bruising in several places or one large area.
A swollen painful calf, or sudden breathlessness with chest pain; venous and arterial thromboembolism is a boxed warning and blood-thinning prophylaxis is recommended.
Blood in stool or urine, vomiting blood, a bleed that will not stop, or a severe headache; fatal bleeding events have occurred and the labels advise considering the risk around surgery and with blood thinners.
Macmillan lists difficulty passing urine, loss of bladder or bowel control and constipation among the signs of spinal cord compression and says to contact the hospital straight away. If you cannot reach anyone, go to A and E and say you have lymphoma and symptoms of spinal cord compression.
Yellowing during or after antibody treatment for a B-cell lymphoma is a reason to be seen the same day and to have liver blood tests, not to wait for the next cycle.
See all on the product pages:BendamustineCentral venous access (port, PICC line)Febrile neutropeniaHepatitis B reactivation before rituximab and other anti-CD20 antibodiesHypogammaglobulinaemia and infection risk after B-cell therapiesLenalidomideMetastatic spinal cord compression (MSCC)NeutropeniaZanubrutinib·Printable cards in the navigator
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