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ORPHA:86854

Splenic marginal zone lymphoma

medium confidenceDisorder

Also known as: SMZL

Publications

2,543

89th percentile

Trials

132

Interventional, condition-specific

Researchers

1,378

Distinct authors in sample

Gene link

—

Readiness

3/6

Stages with a signal

Clinical definition (Orphanet)

Splenic marginal zone lymphoma is a rare, indolent B-cell non-Hodgkin lymphoma characterized by abnormal clonal proliferation of mature B-lymphocytes with involvement in the spleen, bone marrow and, frequently, the blood. It usually presents with , lymphocytosis, anemia and/or thrombocytopenia. Hepatitis C virus and autoimmune manifestations, such as autoimmune hemolytic anemia and autoimmune thrombocytopenia, could be associated.

How rare: 1-9 / 1 000 000 — roughly one to nine people per million. In a city the size of Kolkata, perhaps a few dozen.

Orphanet entry

Cross-references

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

Additional Mondo synonyms (8)

SLVL · marginal zone lymphoma of spleen · marginal zone lymphoma of the spleen · splenic lymphoma with circulating villous lymphocytes · splenic marginal zone B-cell lymphoma · splenic marginal zone B-cell lymphoma with villous lymphocytes · splenic marginal zone lymphoma · splenic marginal zone lymphoma with villous lymphocytes

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

    2,543 matched papers (1,346 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 designationPresent

    10 FDA · 1 EMA designations (6 FDA orphan-indication approvals) — e.g. zanubrutinib Source

  6. Interventional trialPresent

    132 matched on ClinicalTrials.gov (6 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)

11

Designations · 6 with FDA orphan-indication approval

  • FDA zanubrutinibSplenic marginal zone lymphoma · 2020-08-24 · Not FDA Approved for Orphan Indication
  • FDA parsaclisibSplenic marginal zone lymphoma · 2019-09-10 · Not FDA Approved for Orphan Indication
  • FDA obinutuzumabSplenic marginal zone lymphoma · 2017-08-17 · Not FDA Approved for Orphan Indication
  • FDA copanlisibSplenic marginal zone lymphoma · 2017-02-07 · Not FDA Approved for Orphan Indication
  • FDA obinutuzumabSplenic marginal zone lymphoma · 2015-06-11 · Not FDA Approved for Orphan Indication
  • FDA idelalisibSplenic marginal zone lymphoma · 2013-10-15 · Not FDA Approved for Orphan Indication
  • FDA umbralisib (Ukoniq)Splenic marginal zone lymphoma · 2019-04-11
  • FDA lenalidomide (Revlimid)Splenic marginal zone lymphoma · 2017-01-04

Sources: FDA OOPD · EMA orphan designations

Open Targets candidates

11

Drugs / clinical candidates · MONDO_0019462

CTD chemicals (MyDisease.info)

No CTD chemical associations returned for this Mondo ID.

Literature

Is anyone studying this?

2,543

2,543 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.

2,543 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).

1,346 in the last 10 years · medium confidence · 89th percentile (publications denominator)

Phrase hits: 2,543 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,378

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
    Zhang X7 papers · 2026

    Department of Anatomic and Clinical Pathology, Northwell Health, Greenvale.

    Papers in Europe PMC
  2. 02
    Ghia P6 papers · 2026

    Università Vita-Salute San Raffaele Milano Italy.

    Papers in Europe PMC
  3. 03
    Stamatopoulos K6 papers · 2026

    CERTH Thessaloniki Greece.

    Papers in Europe PMC
  4. 04
    Rossi D5 papers · 2026

    The Oncology Institute of Southern Switzerland Bellinzona Switzerland.

    Papers in Europe PMC
  5. 05
    Thieblemont C5 papers · 2026

    Assistance Publique-Hôpitaux de Paris, Hopital Saint-Louis, Hemato-Oncology Unit; Université de Paris, Paris, France.

    Papers in Europe PMC
  6. 06
    Traverse-Glehen A5 papers · 2026

    Division of Pathology, Centre Hospitalier Lyon Sud, Lyon, France.

    Papers in Europe PMC
  7. 07
    Zhang Y5 papers · 2026

    Department of Nephrology, Weifang People's Hospital (First Affiliated Hospital of Shandong Second Medical University), No. 151 Guangwen Street, Kuiwen District, Weifang, Shandong, 261000, China.

    Papers in Europe PMC
  8. 08
    Kalpadakis C4 papers · 2026

    Department of Hematology, University Hospital, University of Crete, Heraklion, Greece.

    Papers in Europe PMC
  9. 09
    Medeiros LJ4 papers · 2025

    Department of Hematopathology The University of Texas MD Anderson Cancer Center Houston Texas USA.

    Papers in Europe PMC
  10. 10
    Wang J4 papers · 2026

    State Key Laboratory of Experimental Hematology, Institute of Hematology and Blood Disease Hospital, National Clinical Research Center for Hematological Disorders, Chinese Academy of Medical Sciences and Peking Union Medical College, Tianjin, China.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

132

interventional trials for this specific condition

132 interventional trials matched this specific condition name; 6 currently recruiting in our sample. 219 trials are registered for marginal zone lymphoma, the broader category — shown separately because they may or may not enrol this specific subtype.

Data as of 11 September 2026

132 interventional trials — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 98.8th percentile).

medium confidence · 98.8th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

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

Broader category: marginal zone lymphoma

219

Interventional trials for the parent category, exclusive of NCT IDs already counted above. Eligibility for this subtype is not guaranteed.

Worth raising with a clinician. How we count trials.

Recruiting under the broader category

Observational and natural-history studies

5 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 8 · after dedupe 8 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 8 · 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 (8)

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 Splenic marginal zone 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.

"Splenic marginal zone lymphoma" OR "marginal zone lymphoma of spleen" OR "marginal zone lymphoma of the spleen" OR "splenic lymphoma with circulating villous lymphocytes" OR "splenic marginal zone B-cell lymphoma" OR "splenic marginal zone B-cell lymphoma with villous lymphocytes" OR "splenic marginal zone lymphoma with villous lymphocytes"

Run this search on Europe PMC

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

"Splenic marginal zone lymphoma" OR "marginal zone lymphoma of spleen" OR "marginal zone lymphoma of the spleen" OR "splenic lymphoma with circulating villous lymphocytes" OR "splenic marginal zone B-cell lymphoma" OR "splenic marginal zone B-cell lymphoma with villous lymphocytes" OR "splenic marginal zone lymphoma with villous lymphocytes"

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: 132 interventional · 5 observational · 0 expanded access. Only interventional studies enter the trial headline.

Parent-category trials query:

"marginal zone lymphoma"

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

Run this search on ClinicalTrials.gov

Synonyms dropped by stoplist: SMZL; SLVL

Confidence reasoning

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

Ingested 2026-07-27T03:12:39.282Z