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ORPHA:86867

Nodal marginal zone B-cell lymphoma

medium confidenceDisorder

Also known as: NMZL

Publications

1,092

91.4th percentile

Trials

137

Interventional, condition-specific

Researchers

1,320

Distinct authors in sample

Gene link

—

Readiness

2/6

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.”

2/6 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 checked

    Not yet enriched from Monarch / HPO

  4. Animal modelNot checked

    Not yet enriched from Monarch / Alliance

  5. Orphan designationNot checked

    FDA/EMA orphan-drug designation not enriched yet

  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)

Not enriched in this build — Monarch phenotype joins were not run for this record.

Animal models (Monarch / Alliance)

Not enriched in this build.

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=5449) is 41.

1,092 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 41 (publications denominator n=5449).

611 in the last 10 years · medium confidence · 91.4th 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 27 July 2026

137 interventional trials — more than 73% of diseases in the trials denominator have none at all (5114 of 7003; 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

Where to find support

We do not yet link condition-specific patient organisations. These umbrella groups support undiagnosed and ultra-rare families:

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