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ORPHA:86867

Nodal marginal zone B-cell lymphoma

medium confidenceDisorder

Also known as: NMZL

Publications

1,092

84.9th percentile

Trials

137

Interventional, condition-specific

Researchers

1,320

Distinct authors in sample

Gene link

—

Readiness

3/6

Stages with a signal

Clinical definition (Orphanet)

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.

How rare: 1-9 / 100 000 — about one to nine people per hundred thousand.

Orphanet entry

Cross-references

Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.

Additional Mondo synonyms (4)

Monocytoid B-cell lymphoma · nodal marginal zone B-cell lymph. · nodal marginal zone B-cell lymphoma · nodal marginal zone lymphoma

Research stages

Trial readiness signals

Where this condition sits on an open-data research pipeline — not how close a treatment is, and not medical advice. Empty stages often mean “not in these databases under this Mondo ID,” not “impossible.”

3/6 stages with a signal

An interventional trial matched this condition name on ClinicalTrials.gov — see trials below.

  1. Gene identifiedNot found

    No GenCC disease–gene assertion in this build

  2. LiteraturePresent

    1,092 matched papers (611 in last 10 years) Source

  3. Phenotype characterisedNot found

    No HPO disease–phenotype associations via Monarch for these Mondo IDs

  4. Animal modelNot found

    No Alliance genotype “model of” associations via Monarch for these Mondo IDs

  5. Orphan designationPartial

    1 FDA · 1 EMA designations (none yet with FDA orphan-indication approval) — e.g. bendamustine hydrochloride Source

  6. Interventional trialPresent

    137 matched on ClinicalTrials.gov (7 recruiting in sample)

Biology

Genes and phenotypes

Gene–disease validity from GenCC, plus phenotypes and animal models joined from Monarch Initiative via Mondo ID — not a clinical diagnosis aid.

Do we know what causes it?

Not yet — the cause hasn't been pinned down in GenCC.

No strong gene–disease assertion joined for this Orphanet entity.

Phenotypes (Monarch / HPO)

None returned for this Mondo ID. That often means “not linked under this ID,” not “no clinical features.”

Animal models (Monarch / Alliance)

None returned for this Mondo ID. Empty here is not proof that no model organism work exists under another name or gene.

Monarch fetch 2026-07-29

Therapies

Designations, candidates, and chemicals

FDA OOPD and EMA orphan designations, Open Targets clinical candidates, and CTD chemical associations via MyDisease.info. These never change the interventional-trial headline.

Orphan designation (FDA · EMA)

2

Designations · no FDA orphan-indication approval yet

  • FDA bendamustine hydrochloride (Treanda)follicular lymphoma lymphoplasmacytic lymphoma Mucosa-associated lymphoma Splenic marginal zone lymphoma Nodal Marginal Zone Lymphoma Extranodal marginal zone B-cell lymphoma · 2013-11-26
  • EMA IdelalisibTreatment of nodal marginal-zone lymphoma · 05/08/2013 · WithdrawnEMA designation

Sources: FDA OOPD · EMA orphan designations

Open Targets candidates

9

Drugs / clinical candidates · MONDO_0019465

CTD chemicals (MyDisease.info)

No CTD chemical associations returned for this Mondo ID.

Literature

Is anyone studying this?

1,092

1,092 papers have been published on this condition. That is a real research literature — still far smaller than common diseases (breast cancer: over 700,000 papers). Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=3967) is 59.

1,092 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 59 (publications denominator n=3967).

611 in the last 10 years · medium confidence · 84.9th percentile (publications denominator)

Phrase hits: 1,092 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,320

Distinct author names in 200 sampled papers — named people below.

Who's working on it?

People publishing on this condition (sampled Europe PMC records). Affiliation is the most recent found in that sample.

  1. 01
    Quintanilla-Martinez L8 papers · 2026

    Institute of Pathology and Neuropathology, Eberhard Karls University of Tübingen and Comprehensive Cancer Center, Tübingen University Hospital, Tübingen, Germany.

    Papers in Europe PMC
  2. 02
    Epperla N5 papers · 2025

    Division of Hematology, Department of Medicine, Ohio State University Comprehensive Cancer Center, Columbus, Ohio, USA.

    Papers in Europe PMC
  3. 03
    Jaffe ES4 papers · 2024

    National Institutes of Health, Bethesda, MD.

    Papers in Europe PMC
  4. 04
    Laurent C4 papers · 2026

    Toulouse Center of Research on Cancer-Oncopole, CHU Toulouse, CRCT Inserm U1037, Toulouse, France.

    Papers in Europe PMC
  5. 05
    Zhang X4 papers · 2025

    Department of Hematology, Taizhou Central Hospital (Taizhou University Hospital), Taizhou, Zhejiang, People's Republic of China.

    Papers in Europe PMC
  6. 06
    Anampa-Guzmán A3 papers · 2025

    Department of Medicine, Roswell Park Comprehensive Cancer Center, Buffalo, New York, USA.

    Papers in Europe PMC
  7. 07
    Annibali O3 papers · 2026

    Division of Hematology, Stem Cell Transplantation, University Campus Bio-Medico, Roma, Italy.

    Papers in Europe PMC
  8. 08
    Annunzio K3 papers · 2025

    Division of Hematology, Department of Medicine, Ohio State University Comprehensive Cancer Center, Columbus, Ohio, USA.

    Papers in Europe PMC
  9. 09
    Barta SK3 papers · 2025

    Department of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.

    Papers in Europe PMC
  10. 10
    Bartlett NL3 papers · 2025

    Department of Medicine, Siteman Cancer Center, Washington University School of Medicine, St. Louis, Missouri, USA.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

137

interventional trials for this specific condition

137 interventional trials matched this specific condition name; 7 currently recruiting in our sample.

Data as of 11 September 2026

137 interventional trials — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 98.9th percentile).

medium confidence · 98.9th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

137 interventional trials matched after quoted-phrase search and title/condition post-filter.

Observational and natural-history studies

2 observational studies match this condition. These do not test a treatment and are not counted in the interventional-trial headline, but they are genuine research: natural-history work often defines the endpoints needed for a future rare-disease trial, and families may be able to enroll.

Recruiting or not-yet-recruiting

Open the complete matched search on ClinicalTrials.gov

Other registries (secondary)

Broader net from EU CTIS, ISRCTN, and ICTRP when available — deduped against ClinicalTrials.gov IDs already counted above. Dual-model LLM relevance gates what we keep. These rows are not added to the interventional headline.

raw 7 · after dedupe 7 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 7 · dropped 0 · fetched 2026-07-30

Source notes: ictrp: Error: ICTRP public search unavailable (WHO portal is SPA-only; SOAP needs partnership). Tried: https://apps.who.int/tri

No secondary-registry studies passed dual-model relevance for this condition name (after dedupe).

Uncertain / not reviewed (7)

Where to find support

Condition-specific patient organisations, when Orphanet lists them, are on the disease’s Orphanet page. We also link umbrella groups that support undiagnosed and ultra-rare families.

Orphanet entry for Nodal marginal zone B-cell lymphoma — check Associations / patient organisations on that page.

India — NPRD

Last verified 2026-07-26

This ORPHAcode is not on our curated NPRD list (direct or Mondo-parent match). That does not decide clinical eligibility; families in India should ask a notified Centre of Excellence about current coverage.

Hand-curated for this project. ORPHAcode mappings are best-effort and may be incomplete or imprecise for umbrella categories. Parent (Mondo) matches mean the policy lists a broader category — confirm eligibility with a Centre of Excellence. Financial entitlements summarised from public policy statements and may change. This is not official government guidance.

How we counted this

Europe PMC query (preferred label + any corrected label + Orphanet and Mondo exact synonyms, stoplisted; unioned with resolved MeSH labels when available). UMLS / OMIM / NCIT cross-references are stored on the overview but are not added to the query string.

"Nodal marginal zone B-cell lymphoma" OR "Monocytoid B-cell lymphoma" OR "nodal marginal zone B-cell lymph." OR "nodal marginal zone lymphoma"

Run this search on Europe PMC

ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):

"Nodal marginal zone B-cell lymphoma" OR "Monocytoid B-cell lymphoma" OR "nodal marginal zone B-cell lymph." OR "nodal marginal zone lymphoma"

Interventional trials matched via: phrase (mesh = registered under a MeSH descriptor no name phrase would catch; recall-expansion = gene / selected parent terms used only for trials).

Study-type breakdown: 137 interventional · 2 observational · 0 expanded access. Only interventional studies enter the trial headline.

Query health: ok — strategies attempted: phrase; with hits: phrase

Run this search on ClinicalTrials.gov

Synonyms dropped by stoplist: NMZL

Confidence reasoning

  • Preferred label is multi-word and distinctive
  • 1 synonym(s) dropped by stoplist (may under-count)
  • No label/synonym collisions with other diseases in this corpus

Ingested 2026-07-27T03:13:33.668Z