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nodal marginal zone B-cell lymphoma - Clinical Profile, Treatments & Trials | Kisho | Kisho

Rare Disease

nodal marginal zone B-cell lymphoma

MONDO:0019465

Also known as:Monocytoid B-cell lymphomaNMZLnodal marginal zone B-cell lymph.

Find Clinical TrialsTrials

Overview

Nodal marginal zone B-cell lymphoma is a rare, indolent B-cell non-Hodgkin lymphoma, characterized by abnormal clonal proliferation of mature B-lymphocytes with involvement of the lymph nodes, sometimes the bone marrow, and rarely the blood. Clinically it presents with disseminated peripheral, abdominal and/or thoracic lymphadenopathy. Cytopenia and bulky tumors (greater than 5 cm) are rare. Association with Hepatitis C virus and chronic inflammation has been reported.

Treatments3 FDA approved

Clinical Trials17 registered · 6 recruiting

Diagnosis

Biomarker and diagnostic research for nodal marginal zone B-cell lymphoma has been reported in the published literature.

Treatment

Compiled from FDA-approved drug records, orphan drug designations, and FDA Adverse Event Reporting System (FAERS) safety data. GeneReviews management guidance is included where available.

3 FDA-approved treatments are available for nodal marginal zone B-cell lymphoma, including ukoniq (UKONIQ, approved 2021), LENALIDOMIDE (REVLIMID, approved 2005), and IBRUTINIB (IMBRUVICA, approved 2022). An additional 11 compounds hold orphan drug designation.

Approved Therapies

Brand Name	Generic Name	Mechanism	Approved

Prognosis

Estimated prevalence: 1-9 in 100,000 (Uncommon).

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

17 clinical trials registered, 6 recruiting. Interventions under study include drug therapy, other interventions, procedural interventions, and biologic therapy. Pipeline includes 7 PHASE2, 5 PHASE1. Research is sponsored by a mix of industry and academic institutions.

NCT ID	Title	Phase	Sponsor	Status
[NCT06859008](https://clinicaltrials.gov/study/NCT06859008)

Data assembled from 5 of 12 sources · Last updated Sep 19, 2026, 2:58 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

Orphanet

European rare disease database

Orphanet:86867

GARD

Genetic and Rare Diseases Info Center

GARD:19075

Questions for Your Care Team

→What are the benefits and potential side effects of UKONIQ for managing nodal marginal zone B-cell lymphoma?
→Are any of the 17 active clinical trials a good fit for my situation?
→Are there specialists or centers of excellence for this condition in my area?
→Is there a patient registry I should consider joining?

← Back to All Diseases

Brand Name	Generic Name	Sponsor	Designated	Exclusivity End	Designation Status
odronextamab	odronextamab	Regeneron Pharmaceuticals, Inc.	2025	—	Designated
tafasitamab-cxix	tafasitamab-cxix	Incyte Corporation	2024	—	Designated
Breyanzi	lisocabtagene maraleucel	Juno Therapeutics, Inc., a Bristol-Myers Squibb Company	2023	—	Designated (drug approved for other indication)
Brukinsa	zanubrutinib	BeOne Medicines USA, Inc.	2020	2028	Designated (drug approved for other indication)
axicabtagene ciloleucel	axicabtagene ciloleucel	Kite Pharma, Inc.	2020	—	Designated
parsaclisib	parsaclisib	Incyte Corporation	2019	—	Designated
Ukoniq	umbralisib	TG Therapeutics, Inc.	2019	2022	Designated (drug approved for other indication)
copanlisib	copanlisib	Bayer HealthCare Pharmaceuticals, Inc.	2017	—	Withdrawn
Bendeka	bendamustine for 50ml admixture	Eagle Pharmaceuticals, Inc.	2014	2022	Designated (drug approved for other indication)
idelalisib	idelalisib	Gilead Sciences, Inc.	2013	—	Designated
ublituximab	ublituximab	TG Therapeutics, Inc.	2013	—	Withdrawn

Research Type	Count	% of Total
Patient case studies	19	54%
Research summaries	7	20%
Other research	2	6%
Laboratory research	2	6%
Disease patterns and progression	2	6%
Testing and diagnosis research	1	3%
Clinical study results	1	3%
New treatment approaches	1	3%

Kisho

nodal marginal zone B-cell lymphoma

Overview

Diagnosis

Treatment

Approved Therapies

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Questions for Your Care Team

Questions for Your Care Team

Orphan Drug Designations

Clinical Trials

Recent Publications

Latest News & Developments

Nodal marginal zone lymphoma: genetic analysis and the differential diagnosis from other small B-cell lymphomas.

BRAF V600E-mutated marginal zone lymphoma may exhibit features that partially overlap with hairy cell leukemia.

Treatment patterns in relapsed/refractory marginal zone lymphoma: real-world evidence from seven countries.

FDA Approves First CAR T-Cell Therapy for Marginal Zone Lymphoma In the US

nodal marginal zone B-cell lymphoma

Overview

Diagnosis

Treatment

Approved Therapies

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

Orphan Drug Designations

Clinical Trials

Recent Publications

Latest News & Developments

Nodal marginal zone lymphoma: genetic analysis and the differential diagnosis from other small B-cell lymphomas.

BRAF V600E-mutated marginal zone lymphoma may exhibit features that partially overlap with hairy cell leukemia.

Treatment patterns in relapsed/refractory marginal zone lymphoma: real-world evidence from seven countries.

FDA Approves First CAR T-Cell Therapy for Marginal Zone Lymphoma In the US