{"schema":"https://ver.cy/schemas/card/1.0.0","id":"vr.tr.hypereosinophilic-syndrome","code":"thing-q2551272","url":"https://ver.cy/models/thing/q2551272/","name":"hypereosinophilic syndrome","alternateNames":["primary hypereosinophilic syndrome","secondary hypereosinophilic syndrome","idiopathic hypereosinophilic syndrome"],"kind":"thing","status":"published","version":"0.1.0-research.1","language":"en","classifiers":{"family":"Thing Registry","category":"Information and virtual systems","entryKind":"thing","plane":"INF","domain":["INF.KNW"],"industry":[],"navPath":"","tags":[],"facets":{}},"whatItIs":"Hypereosinophilic syndrome in this card means idiopathic hypereosinophilic syndrome, characterised by unexplained chronic eosinophilia with organ damage or dysfunction attributable to eosinophils, after recognised reactive, neoplastic and other causes and alternative explanations for the organ injury have been excluded.","purpose":"Enable an agent to recognise the unexplained chronic form of hypereosinophilic syndrome, assess evidence of eosinophil-attributable injury and identify evidence needed for clinician-directed management.","scope":{"in":["Versioned criteria distinguish unexplained chronic eosinophilia from idiopathic hypereosinophilic syndrome.","Blood and tissue eosinophilia records include sampling dates and treatment context.","Evidence supports attribution of organ dysfunction or damage to eosinophilic disease.","Evidence supports exclusion of recognised causes and records uncertainty about competing explanations.","Longitudinal records describe disease activity, accumulated damage, treatment response and relapse."],"out":["The patient's complete identity, medical history and unrelated conditions are outside this model.","Encounter scheduling, billing and episode-of-care administration are outside this model.","Reactive or neoplastic forms of hypereosinophilic syndrome are outside this card's unexplained chronic sense.","Standalone models of eosinophilic leukaemia, parasitic infection and other underlying diseases are outside this model.","Complete models of asthma, eosinophilic gastrointestinal disease and eosinophilic granulomatosis with polyangiitis are outside this model.","Drug manufacturing, prescribing systems and general laboratory procedures are outside this model."],"boundaries":[]},"distinguishingFeatures":["Evidence of eosinophil-attributable organ dysfunction or damage distinguishes the syndrome from an elevated eosinophil count alone.","This card covers the idiopathic form with unexplained chronic eosinophilia, so an established reactive or neoplastic cause places the condition outside its scope.","The recorded diagnostic framework must support the unexplained chronic sense used here, with uncertainty retained where tissue-restricted disease or overlap with named disorders does not clearly fit.","Concurrent eosinophilia does not establish causation when organ injury has a better alternative explanation.","The syndrome, the clinician's diagnostic assertion and the episode of care remain distinct linked entities."],"structure":{"bundles":[{"id":"syndrome-definition-and-boundaries","name":"Syndrome definition and boundaries","description":"Establish what qualifies as HES under an explicitly selected framework.","layers":[{"id":"qualifying-eosinophilia","name":"Qualifying eosinophilia","description":"Assess blood and tissue evidence against the applicable criteria.","findings":[{"id":"blood-and-tissue-qualification","name":"Blood and tissue qualification","description":"Record criterion thresholds, observation intervals and tissue routes without assuming that all frameworks agree.","questions":[{"text":"Which issuing group and revision define the blood threshold, repeat-observation interval and tissue criteria being applied?","id":"blood-and-tissue-qualification-q01","kind":"provenance"},{"text":"Which dated blood counts or tissue observations satisfy each criterion, and were they obtained before eosinophil-suppressing treatment?","id":"blood-and-tissue-qualification-q02","kind":"measurement"},{"text":"How does this framework handle organ-threatening presentations before the usual observation interval is complete?","id":"blood-and-tissue-qualification-q03","kind":"boundary"}]}]},{"id":"syndrome-and-code-identity","name":"Syndrome and code identity","description":"Separate clinical qualification from terminology and coding.","findings":[{"id":"diagnostic-and-terminology-alignment","name":"Diagnostic and terminology alignment","description":"Preserve the distinction between HES, isolated hypereosinophilia and framework-dependent overlap categories.","questions":[{"text":"What evidence establishes eosinophil-attributable dysfunction rather than hypereosinophilia without attributable injury?","id":"diagnostic-and-terminology-alignment-q01","kind":"definition"},{"text":"Does this framework include the observed organ-restricted or overlap presentation within HES?","id":"diagnostic-and-terminology-alignment-q02","kind":"boundary"},{"text":"Which verified ICD-10 national modification, ICD-11 release or SNOMED CT edition maps to this syndrome and subtype?","id":"diagnostic-and-terminology-alignment-q03","kind":"provenance"}]}]}]},{"id":"causal-classification","name":"Causal classification","description":"Determine what drives eosinophilia and how confidently a subtype can be assigned.","layers":[{"id":"reactive-causes-and-mimics","name":"Reactive causes and mimics","description":"Evaluate exposures and associated disorders before assigning unexplained disease.","findings":[{"id":"secondary-cause-assessment","name":"Secondary cause assessment","description":"Track the completeness and limitations of investigations for alternative drivers.","questions":[{"text":"Which medications, travel or residence exposures, infections and associated inflammatory or malignant disorders have been assessed?","id":"secondary-cause-assessment-q01","kind":"provenance"},{"text":"Which possible causes remain untested, and which were assessed with tests whose sensitivity or timing limits exclusion?","id":"secondary-cause-assessment-q02","kind":"boundary"},{"text":"Does the identified condition explain eosinophilia, organ injury, both or neither?","id":"secondary-cause-assessment-q03","kind":"definition"}]}]},{"id":"clonal-and-lymphocytic-evidence","name":"Clonal and lymphocytic evidence","description":"Capture lineage-specific investigations and their implications for classification.","findings":[{"id":"molecular-and-cellular-subtyping","name":"Molecular and cellular subtyping","description":"Distinguish evidence of a myeloid process from an aberrant T-cell-associated process. [ASH clinical approach, 2022](https://ashpublications.org/hematology/article/2022/1/47/493536/Approach-to-the-patient-with-suspected)","questions":[{"text":"What marrow morphology, cytogenetic or molecular evidence supports a myeloid neoplasm, including testing for FIP1L1::PDGFRA where indicated?","id":"molecular-and-cellular-subtyping-q01","kind":"measurement"},{"text":"What immunophenotypic, clonality and functional evidence supports a lymphocytic variant, and what remains inferential?","id":"molecular-and-cellular-subtyping-q02","kind":"measurement"},{"text":"Should the result revise the HES subtype and link to a separately classified underlying neoplasm?","id":"molecular-and-cellular-subtyping-q03","kind":"action"}]}]}]},{"id":"organ-injury-and-urgency","name":"Organ injury and urgency","description":"Represent the distribution, attribution and immediate consequences of organ involvement.","layers":[{"id":"organ-attribution","name":"Organ attribution","description":"Connect symptoms and objective dysfunction to evidence of eosinophilic injury.","findings":[{"id":"organ-specific-evidence-map","name":"Organ-specific evidence map","description":"Maintain separate assessments for cardiac, pulmonary, neurological, cutaneous, gastrointestinal and other involvement.","questions":[{"text":"Which organs have symptoms, objective dysfunction, imaging abnormalities or pathological evidence, and on what dates?","id":"organ-specific-evidence-map-q01","kind":"measurement"},{"text":"What supports eosinophilic causation in each organ, and which competing explanations have been considered?","id":"organ-specific-evidence-map-q02","kind":"boundary"}]}]},{"id":"organ-threatening-complications","name":"Organ-threatening complications","description":"Record findings that change the urgency of clinical review.","findings":[{"id":"urgent-complication-assessment","name":"Urgent complication assessment","description":"Capture suspected cardiac injury, thrombosis and severe respiratory or neurological involvement. [French guidelines, 2023](https://pmc.ncbi.nlm.nih.gov/articles/PMC10148979/)","questions":[{"text":"Is there evidence of myocardial injury, intracardiac thrombus, embolism, respiratory compromise or acute neurological deficit?","id":"urgent-complication-assessment-q01","kind":"measurement"},{"text":"Which findings require urgent escalation under the applicable clinical pathway, and has clinician review occurred?","id":"urgent-complication-assessment-q02","kind":"action"}]}]}]},{"id":"course-and-response","name":"Course and response","description":"Track the syndrome over time without conflating laboratory change, clinical activity and permanent damage.","layers":[{"id":"activity-trajectories","name":"Activity trajectories","description":"Align eosinophil measurements with symptoms and organ assessments.","findings":[{"id":"concordant-and-discordant-trajectories","name":"Concordant and discordant trajectories","description":"Make discrepancies between laboratory and clinical observations explicit.","questions":[{"text":"How do absolute eosinophil counts, symptoms and organ-function measurements change across the same observation period?","id":"concordant-and-discordant-trajectories-q01","kind":"measurement"},{"text":"When these trajectories disagree, what evidence distinguishes ongoing disease, treatment effects, another condition and residual damage?","id":"concordant-and-discordant-trajectories-q02","kind":"boundary"}]}]},{"id":"response-relapse-and-populations","name":"Response, relapse and populations","description":"Define longitudinal outcomes and the populations to which supporting estimates apply.","findings":[{"id":"outcome-definitions","name":"Outcome definitions","description":"Use explicit response and relapse definitions rather than assuming a universal staging system.","questions":[{"text":"Which criteria define haematological response, clinical response, remission and relapse for this assessment?","id":"outcome-definitions-q01","kind":"definition"},{"text":"What follow-up interval and observations support the current state, and what irreversible deficits remain?","id":"outcome-definitions-q02","kind":"measurement"}]},{"id":"population-applicability","name":"Population applicability","description":"Contextualise epidemiology and prognosis by case definition and study population.","questions":[{"text":"For any prevalence or incidence estimate, what were the population, geography, observation year, denominator and HES case definition?","id":"population-applicability-q01","kind":"provenance"},{"text":"Does the evidence apply to this age group and causal subtype, or is applicability uncertain?","id":"population-applicability-q02","kind":"boundary"}]}]}]},{"id":"management-decision-evidence","name":"Management decision evidence","description":"Represent prerequisites and outcomes of clinician-directed intervention.","layers":[{"id":"intervention-eligibility","name":"Intervention eligibility","description":"Connect potential interventions to verified indications and individual evidence.","findings":[{"id":"subtype-and-safety-prerequisites","name":"Subtype and safety prerequisites","description":"Record decision-relevant molecular findings, prior responses and infectious exposure assessment.","questions":[{"text":"Which verified subtype, molecular result, organ manifestation or prior response supports the proposed intervention?","id":"subtype-and-safety-prerequisites-q01","kind":"action"},{"text":"Before systemic corticosteroids, how has relevant Strongyloides exposure or infection risk been assessed and addressed by the treating team?","id":"subtype-and-safety-prerequisites-q02","kind":"action"},{"text":"Which current guideline or jurisdiction-specific product indication supports eligibility, including age and subtype restrictions?","id":"subtype-and-safety-prerequisites-q03","kind":"provenance"}]}]},{"id":"treatment-linked-reassessment","name":"Treatment-linked reassessment","description":"Determine whether intervention is meeting its intended clinical objectives.","findings":[{"id":"benefit-toxicity-and-reclassification","name":"Benefit, toxicity and reclassification","description":"Link outcomes to exposure and identify reasons for renewed diagnostic review.","questions":[{"text":"What changes in organ function, symptoms, eosinophil burden and treatment toxicity followed each intervention?","id":"benefit-toxicity-and-reclassification-q01","kind":"measurement"},{"text":"What evidence would prompt clinician review of treatment failure, adherence, a missed driver or revised subtype?","id":"benefit-toxicity-and-reclassification-q02","kind":"action"},{"text":"Which monitoring and reassessment decisions are documented, with responsible clinician and review date?","id":"benefit-toxicity-and-reclassification-q03","kind":"action"}]}]}]},{"id":"evidence-and-external-alignment","name":"Evidence and external alignment","description":"What the world already says about this thing, gathered so the model can be checked against it.","layers":[{"id":"reported-evidence","name":"Reported evidence","description":"Findings from the breadth pass, kept separate from the structural claims.","findings":[{"id":"evidence-confidence-notes","name":"Check these first","description":"Recalled without web access and unsourced; every item is a lead to verify.","questions":[{"text":"Which of these check these first hold for the sense of hypereosinophilic syndrome this model covers, and on what evidence?","id":"evidence-confidence-notes-q01","kind":"provenance"}]},{"id":"evidence-kinds","name":"Kinds and varieties","description":"Recalled without web access and unsourced; every item is a lead to verify.","questions":[{"text":"Which of these kinds and varieties hold for the sense of hypereosinophilic syndrome this model covers, and on what evidence?","id":"evidence-kinds-q01","kind":"provenance"}]},{"id":"evidence-identifiers","name":"Identifiers and schemes","description":"Recalled without web access and unsourced; every item is a lead to verify.","questions":[{"text":"Which of these identifiers and schemes hold for the sense of hypereosinophilic syndrome this model covers, and on what evidence?","id":"evidence-identifiers-q01","kind":"provenance"}]},{"id":"evidence-standards-and-regulation","name":"Standards and regulation","description":"Recalled without web access and unsourced; every item is a lead to verify.","questions":[{"text":"Which of these standards and regulation hold for the sense of hypereosinophilic syndrome this model covers, and on what evidence?","id":"evidence-standards-and-regulation-q01","kind":"provenance"}]},{"id":"evidence-real-world-use","name":"Real-world use","description":"Recalled without web access and unsourced; every item is a lead to verify.","questions":[{"text":"Which of these real-world use hold for the sense of hypereosinophilic syndrome this model covers, and on what evidence?","id":"evidence-real-world-use-q01","kind":"provenance"}]},{"id":"evidence-measurements","name":"Typical measurements","description":"Recalled without web access and unsourced; every item is a lead to verify.","questions":[{"text":"Which of these typical measurements hold for the sense of hypereosinophilic syndrome this model covers, and on what evidence?","id":"evidence-measurements-q01","kind":"provenance"}]},{"id":"evidence-failure-modes-and-hazards","name":"Failure modes and hazards","description":"Recalled without web access and unsourced; every item is a lead to verify.","questions":[{"text":"Which of these failure modes and hazards hold for the sense of hypereosinophilic syndrome this model covers, and on what evidence?","id":"evidence-failure-modes-and-hazards-q01","kind":"provenance"}]},{"id":"evidence-regional-variation","name":"Regional variation","description":"Recalled without web access and unsourced; every item is a lead to verify.","questions":[{"text":"Which of these regional variation hold for the sense of hypereosinophilic syndrome this model covers, and on what evidence?","id":"evidence-regional-variation-q01","kind":"provenance"}]},{"id":"evidence-neighbours","name":"Neighbouring kinds and how to tell them apart","description":"Recalled without web access and unsourced; every item is a lead to verify.","questions":[{"text":"Which of these neighbouring kinds and how to tell them apart hold for the sense of hypereosinophilic syndrome this model covers, and on what evidence?","id":"evidence-neighbours-q01","kind":"provenance"}]}]}]}]},"agentConduct":{"may":["Explain hypereosinophilic syndrome in general terms from current clinical sources.","Help a patient prepare questions for their haematologist.","Record diagnoses and results in a clinical system it is authorized to use."],"mustNot":["Diagnose or exclude the syndrome from a blood count or symptoms a user reports.","Advise starting, stopping or changing treatment such as steroids or targeted drugs.","Share a patient's diagnosis or test results beyond the care team without consent.","Promote unproven remedies for a rare disease to worried patients."],"requiresHuman":["Any treatment decision for a patient with suspected hypereosinophilia.","Telling a patient about a diagnosis or prognosis."]},"ethics":{"considerations":["Rare diseases are easily missed or misdiagnosed, and delays can cause lasting organ damage.","Patients with rare conditions are targets for misleading treatment offers."],"affectedParties":["Patients and their families","Clinicians","Rare-disease communities"]},"owners":{"steward":"The treating clinician answers for diagnosis and care; the patient owns the decisions about their body.","roles":[],"masterSystems":["Patient health records","Rare-disease registries"]},"relations":[{"target":"Q2305206","type":"parent","note":"registry parent class"}],"interaction":{"identity":{"applicability":"required","items":["Vercy registry: vr.tr.hypereosinophilic-syndrome","Wikidata: Q2551272 (https://www.wikidata.org/wiki/Q2551272)"]},"properties":{"applicability":"not-applicable","items":["Absolute eosinophil count: cells/µL or ×10^9/L, with specimen date and reference interval","Persistence and recurrence: Dated qualifying observations and intervals in days","Tissue eosinophilic involvement: Organ, biopsy site, eosinophil density or qualitative pathology assessment, method and date","Diagnostic framework: Issuing group, document, publication year, revision and criterion-level assessment","Diagnostic certainty: Suspected, established under named criteria, unresolved or refuted","Causal subtype: Framework-specific subtype, supporting evidence and unresolved alternatives","Organ injury attribution: Affected organ linked to evidence, competing causes and clinician attribution","Activity and residual damage: Active, controlled, relapsing or uncertain; residual damage recorded separately","Treatment exposure at assessment: Linked intervention, dose, timing and assessment date"]},"recognition":{"applicability":"not-applicable","items":[]},"capabilities":{"applicability":"required","items":["Compare available evidence with a named diagnostic framework and identify unmet or unassessed criteria","Assemble a subtype-specific investigation checklist for clinician review","Flag documented organ-threatening findings for urgent clinical assessment","Track eosinophil measurements alongside organ function and symptoms","Reassess causal classification when molecular, pathological or exposure evidence changes","Prepare an evidence-linked longitudinal summary distinguishing response, relapse and residual damage"]},"hazards":{"applicability":"required","items":["Eosinophils can damage the heart, lungs, skin and nerves if the condition is untreated.","Blood clots and heart failure are serious complications."]},"interfaces":{"applicability":"required","items":["The International Classification of Diseases, Tenth Revision, Clinical Modification, maintained by the United States National Center for Health Statistics, includes category D72.11 for hypereosinophilic syndrome."]},"context":{"applicability":"required","items":["Hypereosinophilic syndrome is encountered in specialist evaluation of persistent eosinophilia associated with organ injury or dysfunction.","The term is used in clinical records, diagnostic investigations and research to distinguish eosinophil-attributable disease from an isolated elevated cell count."]}},"sources":[{"title":"Wikidata item Q2551272: hypereosinophilic syndrome","url":"https://www.wikidata.org/wiki/Q2551272","note":"identity and sense of the item"},{"title":"Wikipedia: Hypereosinophilic syndrome","url":"https://en.wikipedia.org/wiki/Hypereosinophilic_syndrome","note":"general description of the item"}],"openQuestions":["Which diagnostic framework and revision should govern this registry entry, particularly repeat-count intervals and tissue-restricted HES?","How should this catalogue represent overlap with EGPA and organ-specific eosinophilic disorders while preserving one source of truth for each registered condition?","Which exact ICD-10 national modification codes, ICD-11 entities and SNOMED CT concepts match the selected umbrella and subtype definitions?","Which population-specific incidence, prevalence and outcome estimates remain defensible after accounting for changing case definitions and referral bias?","Which validated activity, damage, response and relapse measures are suitable across HES subtypes, and where are subtype-specific assessments required?"],"resources":{"spec":"/models/things/publications/thing-q2551272/spec.json"},"provenance":{"origin":"thing registry research (pass 2)","builtFrom":["models/things/publications/thing-q2551272/spec.json"],"providers":["Codex"],"researchStatus":"unreviewed","generatedAt":"2026-09-12T10:23:00Z","builder":"tools/build_cards.py@1.0.0"},"completeness":{"sections":{"classifiers":"filled","whatItIs":"filled","purpose":"filled","distinguishingFeatures":"filled","structure":"filled","agentConduct":"filled","ethics":"filled","owners":"filled","relations":"filled","interaction.identity":"filled","interaction.properties":"not-applicable","interaction.recognition":"not-applicable","interaction.capabilities":"filled","interaction.hazards":"filled","interaction.interfaces":"filled","interaction.context":"filled","sources":"filled"},"notes":{"_gate":"Published through the thing publication gate 1.0 on 2026-10-07T12:16:31+00:00: completeness 1.00; sense: Wikidata description; 2 live sources; review by codex: pass","interaction.properties":"Plane INF: no invented physical properties.","interaction.recognition":"This clinical syndrome has no distinctive visual appearance and requires laboratory findings and clinical assessment for identification."},"score":1.0}}