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ORPHA:1959

Evans syndrome

medium confidenceDisorder

Also known as: Immune pancytopenia · Autoimmune hemolytic anemia and autoimmune thrombocytopenia

Publications

2,373

89.4th percentile

Trials

8

Interventional, condition-specific

Researchers

1,402

Distinct authors in sample

Gene link

—

Readiness

3/6

Stages with a signal

Clinical definition (Orphanet)

A rare chronic hematologic disorder characterized by the simultaneous or sequential association of autoimmune hemolytic anemia (AIHA; a disorder in which auto-antibodies are directed against red blood cells causing anemia of varying degrees of severity) with immune thrombocytopenic purpura (ITP; a coagulation disorder in which auto-antibodies are directed against platelets causing hemorrhagic episodes) and occasionally autoimmune neutropenia, in the absence of a known underlying .

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 (4)

Evans' syndrome · autoimmune hemolytic anaemia and autoimmune thrombocytopenia · autoimmune hemolytic anemia and autoimmune thrombocytopenia · immune pancytopenia

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

    2,373 matched papers (1,484 in last 10 years) Source

  3. Phenotype characterisedPresent

    13 HPO annotations (e.g. Bruising susceptibility; Fatigue; Autoimmune thrombocytopenia) Source

  4. Animal modelNot found

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

  5. Orphan designationNot found

    No FDA or EMA orphan-drug designation matched this disease via UMLS or preferred name Source

  6. Interventional trialPresent

    8 matched on ClinicalTrials.gov (4 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)

13

Associated phenotypes · MONDO:0016030

  • Bruising susceptibility
  • Fatigue
  • Autoimmune thrombocytopenia
  • Pallor
  • Muscle weakness

Showing 5 of 13 — open Monarch for the full list.

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)

No designation matched this disease via UMLS or preferred name on the FDA OOPD mirror or EMA orphan register. Absence here is not proof that none exists under another wording.

Open Targets candidates

3

Drugs / clinical candidates · MONDO_0016030

CTD chemicals (MyDisease.info)

2 associated chemicals. Therapeutic evidence is listed first when present — not a treatment recommendation.

  • Penicillamine · marker/mechanism
  • Ramipril · marker/mechanism

MyDisease.info · MONDO:0016030

Literature

Is anyone studying this?

2,373

2,373 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.

2,373 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).

1,484 in the last 10 years · medium confidence · 89.4th percentile (publications denominator)

Phrase hits: 2,373 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,402

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 Y6 papers · 2026

    Department of Neurology, The Second Hospital of Hebei Medical University, Shijiazhuang, Hebei, China.

    Papers in Europe PMC
  2. 02
    Aladjidi N5 papers · 2026

    CEREVANCE, CEREDIH, Pediatric Hemato-Immunology, CIC1401, INSERM CICP, Bordeaux University Hospital, Bordeaux, France.

    Papers in Europe PMC
  3. 03
    Fernandes H4 papers · 2026

    CEREVANCE, CEREDIH, Pediatric Hemato-Immunology, CIC1401, INSERM CICP, Bordeaux University Hospital, Bordeaux, France.

    Papers in Europe PMC
  4. 04
    Grace RF4 papers · 2026

    Dana-Farber/Boston Children's Cancer and Blood Disorders Center, Harvard Medical School, Boston, Massachusetts, USA.

    Papers in Europe PMC
  5. 05
    Leblanc T4 papers · 2026

    CEREVANCE, Pediatric Onco-Hematology, Robert Debré University Hospital, AP-HP, Paris, France.

    Papers in Europe PMC
  6. 06
    Leverger G4 papers · 2026

    CEREVANCE, Pediatric Onco-Hematology, Armand-Trousseau University Hospital, UMR-S398, Sorbonne University, Paris, France.

    Papers in Europe PMC
  7. 07
    Picard C4 papers · 2026

    Study Center for Primary Immunodeficiencies, CEREDIH, Necker-Enfants Malades Hospital, AP-HP, University of Paris Cité, Paris, France.

    Papers in Europe PMC
  8. 08
    Rieux-Laucat F4 papers · 2026

    Laboratory of Human Genetics of Infectious Diseases, Inserm U1163, Imagine Institute, University of Paris Cité, Paris, France.

    Papers in Europe PMC
  9. 09
    Zhang J4 papers · 2026

    Department of Pediatrics, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan, China.

    Papers in Europe PMC
  10. 10
    Almejun MB3 papers · 2025

    IQUIBICEN - Facultad de Ciencias Exactas y Naturales, Universidad de Buenos Aires, Argentina.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

8

interventional trials for this specific condition

8 interventional trials matched this specific condition name; 4 currently recruiting in our sample.

Data as of 11 September 2026

8 interventional trials — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 91.5th percentile).

medium confidence · 91.5th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

8 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 29 · after dedupe 29 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 29 · dropped 0 · fetched 2026-07-29

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 (29)

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 Evans syndrome — 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.

"Evans syndrome" OR "Immune pancytopenia" OR "Autoimmune hemolytic anemia and autoimmune thrombocytopenia" OR "Evans' syndrome" OR "autoimmune hemolytic anaemia and autoimmune thrombocytopenia"

Run this search on Europe PMC

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

"Evans syndrome" OR "Immune pancytopenia" OR "Autoimmune hemolytic anemia and autoimmune thrombocytopenia" OR "Evans' syndrome" OR "autoimmune hemolytic anaemia and autoimmune thrombocytopenia"

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: 8 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

Confidence reasoning

  • Preferred label is short or not clearly distinctive
  • No synonyms dropped by stoplist
  • No label/synonym collisions with other diseases in this corpus

Ingested 2026-07-26T18:37:32.270Z