RARE DISEASERESEARCH ATLAS
Open navigation menu

ORPHA:3260

Idiopathic hypereosinophilic syndrome

low confidenceDisorder

Publications

1,561

Trials

4

Interventional, condition-specific

Researchers

1,068

Distinct authors in sample

Gene link

—

Readiness

2/6

Stages with a signal

Clinical definition (Orphanet)

A rare hematologic disease characterized by eosinophilia without evidence of clonality persisting for at least six months, for which no underlying cause can be identified. The condition is associated with signs of organ damage and dysfunction. Clinical manifestations are highly variable, depending on the organ systems involved, and include rapidly developing, life-threatening cardiovascular or neurological complications.

How rare: How common this is has not been clearly measured.

Orphanet entry

Cross-references

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

Additional Mondo synonyms (1)

hypereosinophilic syndrome, idiopathic, resistant to imatinib, isolated cases, somatic mutation

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

    1,561 matched papers (586 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

    4 matched on ClinicalTrials.gov

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?

1,561

1,561 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.

1,561 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).

586 in the last 10 years · low confidence

Phrase hits: 1,561 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,068

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
    Takahashi H5 papers · 2025

    Division of Respiratory Medicine, Department of Medicine Showa General Hospital Tokyo Japan.

    Papers in Europe PMC
  2. 02
    Zhang J4 papers · 2026

    Queen's Heart Institute, The Queen's Medical Center, Honolulu, Hawaii, USA.

    Papers in Europe PMC
  3. 03
    Li J3 papers · 2025

    Department of Rheumatology and Immunology, Peking University International Hospital, Beijing, China.

    Papers in Europe PMC
  4. 04
    Lübke J3 papers · 2026

    Department of Hematology and Oncology, University Hospital Mannheim, Heidelberg University , ,

    Papers in Europe PMC
  5. 05
    Reiter A3 papers · 2026

    Department of Hematology and Oncology, University Hospital Mannheim, Heidelberg University , ,

    Papers in Europe PMC
  6. 06
    Schwaab J3 papers · 2026

    Department of Hematology and Oncology, University Hospital Mannheim, Heidelberg University , ,

    Papers in Europe PMC
  7. 07
    Singh S3 papers · 2026

    Internal Medicine, Advocate Christ Medical Center, Oak Lawn, USA.

    Papers in Europe PMC
  8. 08
    Wang X3 papers · 2025

    Department of Pathology, The 6th People's Hospital of Chengdu, Chengdu 610051, China.

    Papers in Europe PMC
  9. 09
    Zhang L3 papers · 2026

    Department of Rheumatology and Immunology, Peking University International Hospital, Beijing, China.

    Papers in Europe PMC
  10. 10
    Zhang Y3 papers · 2026

    Department of Rheumatology and Immunology, Peking University International Hospital, Beijing, China.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

4

interventional trials for this specific condition

4 interventional trials matched this specific condition name; none in our sample are currently recruiting. 24 trials are registered for hypereosinophilic syndrome, the broader category — shown separately because they may or may not enrol this specific subtype.

Data as of 27 July 2026

4 interventional trials — more than 73% of diseases in the trials denominator have none at all (5114 of 7003; this disease is at the 86.7th percentile).

low confidence · 86.7th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

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

No currently recruiting studies in the matched set. Open the same search on ClinicalTrials.gov.

Broader category: hypereosinophilic syndrome

24

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

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.

"Idiopathic hypereosinophilic syndrome" OR "hypereosinophilic syndrome, idiopathic, resistant to imatinib, isolated cases, somatic mutation"

Run this search on Europe PMC

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

"Idiopathic hypereosinophilic syndrome" OR "hypereosinophilic syndrome, idiopathic, resistant to imatinib, isolated cases, somatic mutation"

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

Parent-category trials query:

"hypereosinophilic syndrome"

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

Run this search on ClinicalTrials.gov

Confidence reasoning

  • Preferred label is multi-word and distinctive
  • No synonyms dropped by stoplist
  • No label/synonym collisions with other diseases in this corpus
  • Publication count (1561) is extremely high with unknown/missing prevalence — treat as possible over-matching, not proven research intensity

Ingested 2026-07-26T22:39:43.222Z