Raras
Buscar doenças, sintomas, genes...
Síndrome hipereosinofílico idiopático
ORPHA:3260CID-10 · D47.5CID-11 · 4B03.ZOMIM 607685DOENÇA RARA

Síndrome é um conjunto de sinais e sintomas que define as manifestações clínicas de uma ou várias doenças ou condições clínicas, independentemente da etiologia que as diferencia. Por exemplo, a Síndrome de Raynaud pode ser uma manifestação de várias patologias ou mesmo considerado idiopático quando o estudo do paciente não encontra uma causa plausível. Também a Síndrome meníngea pode ser o reflexo de patologias muito diferentes como hemorragia cerebral, meningite purulenta, tuberculose meníngea ou uma simples meningite vírica. Se os sinais são exclusivamente laboratoriais e não clínicos chama-se de síndrome laboratorial para realçar o fato de que não há sinais ou sintomas clínicos que evidenciem doença.

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

📋

Síndrome hipereosinofílico idiopática é uma doença rara caracterizada por eosinofilia persistente e envolvimento multissistêmico, podendo manifestar-se com colite, hipotonia axial, defeitos ventilatórios restritivos e fibrose endocárdica. A trombose venosa é uma complicação frequente.

Pesquisas ativas
1 ensaio
5 total registrados no ClinicalTrials.gov
Publicações científicas
710 artigos
Último publicado: 2026 Apr

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
Unknown
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Europe
Início
Adult
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: D47.5
Você se identifica com essa condição?
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Entender a doença

Do básico ao detalhe, leia no seu ritmo

Preparando trilha educativa...

Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

🩸
Sangue
11 sintomas
🫃
Digestivo
11 sintomas
🫁
Pulmão
9 sintomas
❤️
Coração
6 sintomas
🧠
Neurológico
5 sintomas
🧬
Pele e cabelo
4 sintomas

+ 36 sintomas em outras categorias

Características mais comuns

90%prev.
Eosinofilia
Muito frequente (99-80%)
55%prev.
Desconforto respiratório
Frequente (79-30%)
55%prev.
Palidez
Frequente (79-30%)
55%prev.
Distensão abdominal
Frequente (79-30%)
55%prev.
Febre
Frequente (79-30%)
55%prev.
Mielodisplasia
Frequente (79-30%)
90sintomas
Muito frequente (1)
Frequente (9)
Ocasional (68)
Sem dados (12)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 90 características clínicas mais associadas, ordenadas por frequência.

EosinofiliaEosinophilia
Muito frequente (99-80%)90%
Desconforto respiratórioRespiratory distress
Frequente (79-30%)55%
PalidezPallor
Frequente (79-30%)55%
Distensão abdominalAbdominal distention
Frequente (79-30%)55%
FebreFever
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico710PubMed
Últimos 10 anos200publicações
Pico202528 papers
Linha do tempo
2026Hoje · 2026🧪 1998Primeiro ensaio clínico📈 2025Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

Genes associados

1 gene identificado com associação a esta condição. Padrão de herança: Not applicable.

PDGFRAPlatelet-derived growth factor receptor alphaDisease-causing germline mutation(s) inAltamente restrito
FUNÇÃO

Tyrosine-protein kinase that acts as a cell-surface receptor for PDGFA, PDGFB and PDGFC and plays an essential role in the regulation of embryonic development, cell proliferation, survival and chemotaxis. Depending on the context, promotes or inhibits cell proliferation and cell migration. Plays an important role in the differentiation of bone marrow-derived mesenchymal stem cells. Required for normal skeleton development and cephalic closure during embryonic development. Required for normal dev

LOCALIZAÇÃO

Cell membraneCell projection, ciliumGolgi apparatus

VIAS BIOLÓGICAS (1)
Signaling by PDGF
EXPRESSÃO TECIDUAL(Ubíquo)
Ovário
217.5 TPM
Fibroblastos
164.2 TPM
Cervix Endocervix
100.0 TPM
Fallopian Tube
89.1 TPM
Útero
77.5 TPM
OUTRAS DOENÇAS (8)
polyps, multiple and recurrent inflammatory fibroid, gastrointestinalidiopathic hypereosinophilic syndromeprimary hypereosinophilic syndromegastrointestinal stromal tumor
HGNC:8803UniProt:P16234

Variantes genéticas (ClinVar)

198 variantes patogênicas registradas no ClinVar.

🧬 PDGFRA: NM_006206.6(PDGFRA):c.583G>C (p.Gly195Arg) ()
🧬 PDGFRA: NM_006206.6(PDGFRA):c.910A>G (p.Lys304Glu) ()
🧬 PDGFRA: NM_006206.6(PDGFRA):c.1951C>G (p.Leu651Val) ()
🧬 PDGFRA: NM_006206.6(PDGFRA):c.3169T>G (p.Ser1057Ala) ()
🧬 PDGFRA: NM_006206.6(PDGFRA):c.412G>A (p.Val138Ile) ()
Ver todas no ClinVar

Classificação de variantes (ClinVar)

Distribuição de 170 variantes classificadas pelo ClinVar.

17
136
17
Patogênica (10.0%)
VUS (80.0%)
Benigna (10.0%)
VARIANTES MAIS SIGNIFICATIVAS
PDGFRA: NM_006206.6(PDGFRA):c.932-13C>A [Conflicting classifications of pathogenicity]
PDGFRA: NM_006206.6(PDGFRA):c.*1778C>A [Conflicting classifications of pathogenicity]
PDGFRA: NM_006206.6(PDGFRA):c.2801A>G (p.Asn934Ser) [Uncertain significance]
PDGFRA: NM_006206.6(PDGFRA):c.1790G>T (p.Arg597Leu) [Uncertain significance]
PDGFRA: NM_006206.6(PDGFRA):c.799C>A (p.Pro267Thr) [Uncertain significance]

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
2Fase 24
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 4 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Síndrome hipereosinofílico idiopático

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

🟢 Recrutando agora

1 pesquisa recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

Outros ensaios clínicos

5 ensaios clínicos encontrados, 1 ativos.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

Timeline de publicações
211 papers (10 anos)
#1

Idiopathic Hypereosinophilic Syndrome Presenting as Acute Encephalopathy Secondary to Watershed Ischemic Stroke.

Annals of Indian Academy of Neurology2026 Mar 05
#2

Idiopathic Hypereosinophilic Syndrome in a Child- Rare Disease with Fulminant Clinical Course.

Indian journal of hematology &amp; blood transfusion : an official journal of Indian Society of Hematology and Blood Transfusion2026 Mar
#3

Laboratory Aspects and Diagnostic Challenges in a Case of Hypereosinophilic Syndrome.

Cureus2026 Jan

Hypereosinophilia is defined by a persistent increase in eosinophil levels and can lead to multi-organ damage with highly variable symptoms. The most common manifestations involve the skin, lungs, and gastrointestinal tract, while cardiac and neurological complications can be particularly severe and sometimes fatal. Within this context, we describe the case of a 35-year-old woman with hypereosinophilic syndrome (HES), whose symptoms began during pregnancy. Three months after giving birth, she contracted SARS-CoV-2, which resolved without complications. However, shortly thereafter, laboratory tests revealed marked leukocytosis accompanied by a rapid clinical deterioration. At admission, the patient displayed severe hypereosinophilia associated with systemic inflammation, liver injury, myocardial necrosis, and multi-organ involvement affecting the kidneys, brain, heart, and lungs. As her neurological condition worsened, she required admission to the intensive care unit, where she unfortunately died four days later. The aim of this case report is to raise awareness among clinicians and laboratory professionals about the crucial role of the laboratory finding in assessing disease severity and guiding the diagnostic challenges of idiopathic syndrome (I-HES). The exclusion of secondary causes confirmed the idiopathic nature of the condition. Finally, the case highlights the importance of early recognition of unexplained eosinophilia and systemic symptoms, as timely intervention may be life-saving in rapidly progressive presentations.

#4

Real-world outcomes after anti-IL-5/anti-IL-5Rα treatment for hypereosinophilic syndrome: Systematic literature review.

The journal of allergy and clinical immunology. Global2026 Jan

Anti-IL-5/anti-IL-5 receptor alpha (anti-IL-5/anti-IL-5Rα) treatments have potential to improve outcomes in hypereosinophilic syndrome (HES), as demonstrated in clinical trials. We sought to understand the real-world outcomes of anti-IL-5/anti-IL-5Rα treatments in patients with HES. This systematic literature review searched English-language articles (November 4, 2015, to October 31, 2023) and conference abstracts (January 1, 2021, to October 31, 2023) from MEDLINE/Embase and select conferences for publications containing outcomes for patients with HES receiving anti-IL-5 (mepolizumab [approved], reslizumab) or anti-IL-5Rα (benralizumab) treatment in the real world. Meta-analyses and clinical trials were excluded. Outcomes included treatment response, blood eosinophil (bEOS) counts, and oral corticosteroid (OCS) receipt. No formal bias assessment was conducted. Five cohort studies (192 participants) and 55 case reports (70 participants) were identified. In cohort studies, median age was 44.0 to 61.8 years; 33.3% to 62.9% were female, and 45.5% to 100% had idiopathic HES. Mean (standard deviation) age in case reports (n = 68/70) was 38.5 (23.7) years; 40.0% (n = 28/70) were female, and 67.2% (n = 39/58) had idiopathic HES. Across 4 cohort studies, most patients had reduced or no HES flares after mepolizumab or benralizumab. In patients from case reports, 13 had HES flares (mepolizumab, n = 12/54; benralizumab, n = 1/13) after anti-IL-5/anti-IL-5Rα initiation. Mean bEOS counts were reduced by 53.0% to 100% across 3 cohort studies and by 95.0% in case reports. Of those receiving benralizumab or mepolizumab, OCS dose was reduced in 49.0% to 100% of patients from cohort studies, and by ≥50% in 29 of 70 patients from case reports. Real-world anti-IL-5/anti-IL-5Rα therapy in HES is associated with fewer flares as well as reductions in bEOS counts and OCS dose, although confirmatory studies in larger populations are needed.

#5

Clinical Utility of 18 F-FDG PET/CT in Patients With Hypereosinophilia : An 11-year Single-center Experience.

Clinical nuclear medicine2026 Feb 01

Hypereosinophilia (HE) is a rare disease with complex and diverse causes. Therapeutic strategies for HE differ because of its diverse causes, demanding advanced diagnostic tools for accurate assessment. This study evaluated the role of 18 F-fluorodeoxyglucose positron emission tomography/computed tomography ( 18 F-FDG PET/CT) in determining the etiology of HE. This retrospective single-center study consecutively included 78 patients with HE who underwent 18 F-FDG PET/CT examinations between 2011 and 2022. The diagnostic value of 18 F-FDG PET/CT in identifying the etiology of HE was assessed. Logistic regression analyses were conducted to determine the clinical and laboratory factors associated with 18 F-FDG PET/CT outcomes. Seventy-eight patients aged 44.58±17.01 years had different conditions: infectious diseases, n=9 [11.54%]; noninfectious inflammatory diseases, n=23 [29.49%]; solid tumors or hematological malignancies, n=16 [20.51%]; lymphocyte-variant HE, n=1 [1.28%]; and idiopathic HE or idiopathic hypereosinophilic syndrome, n=29 [37.18%]. 18 F-FDG PET/CT was used to identify the etiology in 48 patients (61.54%, true positive), whereas secondary causes were ruled out in 21 patients (26.92%), leading to a diagnosis of idiopathic HE. 18 F-FDG PET/CT achieved a diagnostic sensitivity of 92.31%, specificity of 80.77%, and accuracy of 88.46%. The presence of symptoms was positively correlated with true-positive PET/CT results (odds ratio: 2.940, 95% CI: 1.073-8.055), whereas immunoglobulin E level exhibited no association. 18 F-FDG PET/CT showed high sensitivity and accuracy in identifying the etiology of HE, making it a valuable tool for assessing elevated eosinophil counts.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC419 artigos no totalmostrando 196

2026

Idiopathic Hypereosinophilic Syndrome Presenting as Acute Encephalopathy Secondary to Watershed Ischemic Stroke.

Annals of Indian Academy of Neurology
2026

Idiopathic Hypereosinophilic Syndrome in a Child- Rare Disease with Fulminant Clinical Course.

Indian journal of hematology &amp; blood transfusion : an official journal of Indian Society of Hematology and Blood Transfusion
2026

Laboratory Aspects and Diagnostic Challenges in a Case of Hypereosinophilic Syndrome.

Cureus
2025

Fulminant Idiopathic Hypereosinophilic Syndrome Presenting with Cardiac, Neurologic and Haematologic Involvement.

European journal of case reports in internal medicine
2026

Real-world outcomes after anti-IL-5/anti-IL-5Rα treatment for hypereosinophilic syndrome: Systematic literature review.

The journal of allergy and clinical immunology. Global
2025

Refractory erythroderma in idiopathic hypereosinophilic syndrome: A case treated with mepolizumab and abrocitinib.

SAGE open medical case reports
2025

Acute multisystemic thromboembolism manifesting as cerebral infarction, myocardial injury, and quadriparesis: a novel triad of idiopathic hypereosinophilic syndrome.

Frontiers in medicine
2025

Hypereosinophilic syndrome with pulmonary and hepatic involvement mimicking autoimmune disease.

Oxford medical case reports
2026

Clinical Utility of 18 F-FDG PET/CT in Patients With Hypereosinophilia : An 11-year Single-center Experience.

Clinical nuclear medicine
2025

Idiopathic hypereosinophilic syndrome complicated by computed tomography-negative cerebral infarction, strangulated intestinal obstruction, and hepatic portal venous gas: a case report of multi-system involvement.

Quantitative imaging in medicine and surgery
2025

Idiopathic hypereosinophilic syndrome associated with multivessel coronary artery ectasia: a case report.

The Pan African medical journal
2025

Panuveitis Associated With Idiopathic Hypereosinophilic Syndrome.

Journal of vitreoretinal diseases
2025

Idiopathic hypereosinophilic syndrome: Potential pathologic somatic gene variants identified by exome sequencing.

The journal of allergy and clinical immunology. Global
2025

Idiopathic hypereosinophilic syndrome in a female Sprague Dawley rat.

Laboratory animals
2025

A Clinical Conundrum in Patients With a Common Clinical Spectrum but Rare Differentials: Hyper-Immunoglobulin E Syndrome and Idiopathic Eosinophilia.

Cureus
2025

Idiopathic Hypereosinophilic Syndrome Presenting As Eosinophilic Ascites and Pleural Effusion in an Elderly Male: A Rare Clinical Manifestation.

Cureus
2025

Benralizumab in a Patient With Refractory Eosinophilic Endocarditis.

Journal of medical cases
2025

[Efficacy and survival outcomes of patients with lymphocytic variant hypereosinophilic syndrome].

Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi
2025

Eosinophilic lung disease with a tumour lysis-like syndrome.

BMJ case reports
2025

Case Report: Idiopathic hypereosinophilic syndrome presenting with gastrointestinal involvement mimicking IBD: a diagnostic challenge.

Frontiers in medicine
2025

[A Case of Idiopathic Hypereosinophilic Syndrome Treated with Mepolizumab].

Acta medica portuguesa
2025

Clinical and immunological biomarkers in hypereosinophilic syndrome: the second step after diagnostic algorithms.

Frontiers in medicine
2025

An Uncommon Case of Non-Specific Interstitial Pneumonia (NSIP) Associated With Idiopathic Hypereosinophilic Syndrome (HES) Reversed by Mepolizumab.

Respirology case reports
2025

Eosinophilia and gastrointestinal symptoms in Immunosuppression: a classic but overlooked diagnosis.

Revista espanola de enfermedades digestivas
2025

Complete hematological response with imatinib mesylate in a corticosteroid refractory idiopathic hypereosinophilic syndrome in person living with HIV: A case report.

International journal of STD &amp; AIDS
2025

Biologic Agents in Idiopathic Hypereosinophilic Syndrome.

Pharmaceuticals (Basel, Switzerland)
2025

Insights from comparison of serum IgG4 between T2-eosinophilic asthma and eosinophilic granulomatosis with polyangiitis/idiopathic hypereosinophilic syndrome.

Clinical and experimental rheumatology
2025

Refractory Idiopathic Hypereosinophilic Syndrome Presenting with Myocarditis and Responding to Imatinib: A Case Report.

Saudi journal of medicine &amp; medical sciences
2025

68 Ga-FAPI PET/CT in a Patient With Multiple Aneurysms Due to Idiopathic Hypereosinophilic Syndrome.

Clinical nuclear medicine
2024

Idiopathic Hypereosinophilic Syndrome With Cutaneous Manifestations: A Case Report.

Cureus
2025

Pulmonary Arterial Hypertension Associated With Idiopathic Hypereosinophilic Syndrome: Importance of Eosinophilia Control With Steroid Therapy.

CJC open
2025

Myocarditis with neurological and dermatological involvement in idiopathic Hypereosinophilic syndrome: Case report.

Radiology case reports
2024

Wells' Syndrome Associated With Idiopathic Hypereosinophilic Syndrome in a Child: A Case Report.

Cureus
2024

[The evaluation of a patient with eosinophilia].

Nederlands tijdschrift voor geneeskunde
2024

Fulminant eosinophilic myocarditis and refractory ventricular arrhythmias requiring mechanical circulatory support: a case report.

European heart journal. Case reports
2024

Hyper eosinophilic diseases - diagnosis and therapeutic approach - practical position of the Polish working group.

Otolaryngologia polska = The Polish otolaryngology
2025

Chronic eosinophilic leukaemia-Not otherwise specified: Clinical features, genomic insight and therapeutic strategies.

British journal of haematology
2024

A Storm of Polyserositis: Unravelling Multisystem Effusions in Hypereosinophilic Syndrome.

Cureus
2024

Multiple Cerebral Infarcts and Encephalopathy as the First Clinical Manifestations of Hypereosinophilic Syndrome: A Case Report and Narrative Review.

Pulse (Basel, Switzerland)
2025

A rare case of PDGFRA-positive chronic eosinophilic leukemia.

Indian journal of pathology &amp; microbiology
2024

A Challenging Case of Idiopathic Hypereosinophilic Syndrome: An Itching in the Brain.

Cureus
2024

Successful novel use of dupilumab for gastrointestinal involvement of idiopathic hypereosinophilic syndrome: case report and review of the literature.

Clinical journal of gastroenterology
2024

Probable idiopathic hypereosinophilic syndrome: A case report of severe multi-organ eosinophilic involvement in a young male presenting with heart failure.

SAGE open medical case reports
2024

Diagnosis of rare Loffler endocarditis by cardiac ultrasound: a case report and literature review.

Medical ultrasonography
2024

A case report of Löffler's endocarditis with right ventricular outflow obstruction in idiopathic hypereosinophilic syndrome: timely surgical intervention is crucial.

European heart journal. Case reports
2024

Radioclinical Profile of Eosinophilic Lung: A Case Series.

Cureus
2024

An Unusual Case of Immune Complex-Mediated Membranoproliferative Glomerulonephritis as Renal Manifestation of Idiopathic Hypereosinophilic Syndrome: A Case Report and Literature Review.

Medicines (Basel, Switzerland)
2024

Idiopathic hypereosinophilic syndrome presenting with stroke.

Clinical neuropathology
2024

Prognostic factors of idiopathic hypereosinophilic syndrome: A nationwide survey in Japan.

British journal of haematology
2024

Cardiac Magnetic Resonance Imaging Used to Determine a Rare Etiology of a Layered Left Ventricular Apical Thrombus.

Cureus
2024

Not just for lymphoid cells: the role of the noncanonical NF-κB signaling pathway in early and late myelopoiesis with a focus on hypereosinophilic disorders.

Journal of leukocyte biology
2024

Multimodality imaging of eosinophilic myocarditis (loeffler's endocarditis) in a patient with idiopathic hypereosinophilic syndrome.

The international journal of cardiovascular imaging
2024

Hypereosinophilic syndrome: a rare cause of ST-elevation myocardial infarction and thrombus formation on the aortic valve.

BMJ case reports
2024

Concurrent Challenges in Idiopathic Hypereosinophilic Syndrome Complicating Beta-Thalassemia Major: A Case Report.

Cureus
2024

World Health Organization and International Consensus Classification of eosinophilic disorders: 2024 update on diagnosis, risk stratification, and management.

American journal of hematology
2024

Idiopathic Hypereosinophilic Syndrome in a Professional Athlete.

Annals of clinical and laboratory science
2024

Eosinophilia and wheeze: thinking beyond asthma.

Breathe (Sheffield, England)
2024

Co-occurrence of Idiopathic Hypereosinophilic Syndrome in End-Stage Renal Disease Patients Undergoing Maintenance Hemodialysis.

Cureus
2023

ETV6::ABL1-Positive Myeloid Neoplasm: A Case of a Durable Response to Imatinib Mesylate without Additional or Previous Treatment.

International journal of molecular sciences
2024

Severe Idiopathic Hypereosinophilic Syndrome with Eosinophilic Cholecystitis in a Child Treated with Mepolizumab.

Indian journal of pediatrics
2023

Case Study: Personalized Oral Low-dose Naltrexone Titration for Pain Management.

International journal of pharmaceutical compounding
2023

An 85-Year-Old Man with Fever, Dyspnea, and Dry Cough Diagnosed with Idiopathic Hypereosinophilic Syndrome, Successfully Treated with High-Dose Corticosteroids.

The American journal of case reports
2023

A Rare Presentation of Hypereosinophilic Syndrome With Loffler's Endomyocarditis, Encephalopathy, and Multiple Thromboembolic Strokes.

Cureus
2023

Hypereosinophilic Syndrome Endocarditis With Severe Biventricular Failure Complicated by Sepsis: A Challenging Case.

Cureus
2023

Pericardial Effusion and Progressive Bilateral Effusion as Rare Presentations of Idiopathic Hypereosinophilic Syndrome.

Cureus
2023

Successful use of mepolizumab for severe hypereosinophilic vasculitis with c-ANCA positivity in a previously healthy 7-year-old boy.

The journal of allergy and clinical immunology. Global
2023

Low-dose anti-IL 5 treatment in idiopathic hypereosinophilic syndrome: towards a precision medicine approach for remission maintenance.

Orphanet journal of rare diseases
2023

A case of atypical IgG4-related disease presenting hypereosinophilia, polyneuropathy, and liver dysfunction.

Modern rheumatology case reports
2023

Idiopathic Hypereosinophilic Syndrome Presenting with Recalcitrant Oral and Genital Ulcers Responding Well to Thalidomide.

Indian dermatology online journal
2023

Idiopathic hypereosinophilic syndrome presenting as capsular warning syndrome: A case report and literature review.

Medicine
2023

Idiopathic hypereosinophilic syndrome presenting as cardiac tamponade and multiorgan dysfunction.

BMJ case reports
2023

Idiopathic hypereosinophilic syndrome with hepatic sinusoidal obstruction syndrome: A case report and literature review.

World journal of gastrointestinal surgery
2023

Chronic Oro-Genital Ulcerations as a Presenting Feature of Chronic Eosinophilic Leukemia: A Case Report.

Indian dermatology online journal
2023

The First Definition of Pulmonary Component of Hypereosinophilic Syndrome: Bronchial Casts.

Thoracic research and practice
2023

Idiopathic Hypereosinophilic Syndrome: A Case Report.

Cureus
2023

Case Report: Off-label treatment of idiopathic hypereosinophilic syndrome with Omalizumab.

Frontiers in pharmacology
2023

Hypereosinophilic syndrome with cutaneous manifestations successfully treated with Dupilumab as a sole agent: A case report.

The Australasian journal of dermatology
2023

Revisiting a case of idiopathic hypereosinophilic syndrome with novel molecular techniques identifies a second case of a myeloid/lymphoid neoplasm with a SART3::PDGFRB fusion.

British journal of haematology
2023

Cryptococcal Neoformans and Varicella Zoster Meningitis in a Patient With Selective Innate Immunodeficiency: A Case Report.

Cureus
2023

Giant Coronary Artery Aneurysm with Thrombosis Complicated in a Patient with Idiopathic Hypereosinophilic Syndrome.

Yonsei medical journal
2022

Idiopathic Hypereosinophilia: A Multicenter Retrospective Analysis.

Journal of asthma and allergy
2023

Cerebral watershed infarctions as a presentation of idiopathic hypereosinophilic syndrome: a case report.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
2023

The role of flexible bronchoscopy in the diagnostic pathway of children with unexplained peripheral eosinophilia.

Pediatrics international : official journal of the Japan Pediatric Society
2022

Intermittent Soft-Tissue Tumor Enlargement in Episodic Angioedema With Eosinophilia Syndrome.

The American Journal of dermatopathology
2022

Eosinophilic Myocarditis Presenting as Cardiac Tamponade: A Diagnostic Challenge.

European journal of case reports in internal medicine
2022

Anesthetic Management of a Large Right Ventricular Mass due to Idiopathic Hypereosinophilic Syndrome in a Patient Undergoing Cardiac Surgery, a Case Report.

Seminars in cardiothoracic and vascular anesthesia
2022

Case report: Application of non-VKA oral anticoagulants in patient of idiopathic hypereosinophilic syndrome with intracardiac thrombus.

Frontiers in pharmacology
2023

Updates on eosinophilic disorders.

Virchows Archiv : an international journal of pathology
2022

Acute exacerbation of idiopathic hypereosinophilic syndrome following asymptomatic coronavirus disease 2019: a case report.

Journal of medical case reports
2022

The Diagnostic Challenge of Eosinophilic Granulomatosis With Polyangiitis Presenting as Acute Eosinophilic Myocarditis: Case Report and Literature Review.

Frontiers in cardiovascular medicine
2022

A novel treatment workflow of idiopathic hypereosinophilic syndrome: a single-center retrospective cohort study.

Chinese medical journal
2022

Rare Case of a Young Male Presented with Abdominal Pain, Solid Colon Tumors, and Eosinophilia, Followed by Tremendous Thromboembolic Complications and Eventually Diagnosed with Idiopathic Hypereosinophilic Syndrome.

Case reports in medicine
2022

A Diagnosis of Idiopathic Hypereosinophilic Syndrome With Skin Involvement in a Patient Presenting With Lymphadenopathy and Rash.

Cureus
2022

Tumefactive eosinophil-rich non-granulomatous small vessel vasculitis in the cerebrum in a patient with idiopathic hypereosinophilic syndrome.

Neuropathology : official journal of the Japanese Society of Neuropathology
2022

Idiopathic Hypereosinophilic Syndrome Associated With Pulmonary Hypertension in an Infant.

Journal of pediatric hematology/oncology
2022

Hyperleucocytosis as an unusual presentation of hypereosinophilic pneumonitis with acute respiratory distress syndrome.

SAGE open medical case reports
2022

A retrospective cohort study of patients with eosinophilia referred to a tertiary centre.

Danish medical journal
2022

The Neutrophil/Lymphocyte Ratio is an Independent Predictor of All-Cause Mortality in Patients with Idiopathic Hypereosinophilic Syndrome.

Journal of inflammation research
2022

Idiopathic hypereosinophilic syndrome with eosinophilic cellulitis-like cutaneous involvement treated with mepolizumab and dapsone.

JAAD case reports
2022

Clinical analysis of hypereosinophilic syndrome first presenting with asthma-like symptoms.

Annals of medicine
2022

Eosinophilic vasculitis as a manifestation of idiopathic hypereosinophilic syndrome.

The Journal of dermatology
2021

Idiopathic Hypereosinophilic Syndrome Presenting With Embolic Stroke.

Cureus
2022

A Rare Manifestation of IgG4-Related Disease and Concurrent Idiopathic Hypereosinophilic Syndrome as Extensive Gastrointestinal Lesions: A Case Report.

Inflammatory bowel diseases
2021

Eosinophilia characterized by a rare CCT6B mutation and responsive to tyrosine kinase inhibition: Case report and literature review.

Leukemia research reports
2021

Idiopathic hypereosinophilic syndrome presenting as mononeuritis multiplex. Lessons learned after 18-months of follow-up.

Neurologia
2022

Acute Liver Failure Due to Hypereosinophilic Syndrome Accompanied by Duodenal Perforation.

Internal medicine (Tokyo, Japan)
2022

World Health Organization-defined eosinophilic disorders: 2022 update on diagnosis, risk stratification, and management.

American journal of hematology
2021

Recurrent prosthetic valve dysfunction in a patient with idiopathic hypereosinophilic syndrome: A case report and literature review.

Journal of cardiac surgery
2022

Eosinophilic Myocarditis: When Allergies Attack the Heart!

Cardiology in review
2021

Renal Involvement as Rare Acute Tubulointerstitial Nephritis in a Patient with Eosinophilic Disorder Treated with Early Add-on Administration of Mepolizumab.

Internal medicine (Tokyo, Japan)
2021

Idiopathic hypereosinophilic syndrome with intracardiac atypical linear-shaped and floating thrombus presenting as embolic cerebral infarction.

Journal of cardiology cases
2021

Investigating Febrile Polyserositis: An Unusual Case of Idiopathic Hypereosinophilic Syndrome.

European journal of case reports in internal medicine
2021

[Multiple cerebral infarctions in the deep perforator regions in a case of idiopathic hypereosinophilic syndrome].

Rinsho shinkeigaku = Clinical neurology
2021

Purtscher-like retinopathy associated with idiopathic hypereosinophilic syndrome.

Journal francais d'ophtalmologie
2021

Idiopathic Hypereosinophilic Syndrome with Multiple Organ Involvement.

Case reports in oncology
2021

Idiopathic hypereosinophilic syndrome in hemodialysis patients: Case reports.

Medicine
2021

Hypereosinophilic syndrome with multiorgan involvement: an interdisciplinary work-up.

BMJ case reports
2021

Favorable Response to Interferon-α in Infantile-onset Idiopathic Hypereosinophilic Syndrome Complicated by Status Epilepticus During Treatment.

Journal of pediatric hematology/oncology
2021

A systems biology approach for defining the potential molecular framework of idiopathic hypereosinophilic syndrome with cutaneous involvement.

Biochemical and biophysical research communications
2021

Clinical and morphologic spectrum of renal involvement in idiopathic hypereosinophilic syndrome.

Clinical and experimental nephrology
2021

A flare up of idiopathic hypereosinophilic syndrome due to COVID-19.

European heart journal
2020

Idiopathic hypereosinophilic syndrome: A rare diagnosis in children.

Clinical case reports
2020

Idiopathic hypereosinophilic syndrome in remission with benralizumab treatment after relapse with mepolizumab.

Respirology case reports
2020

Acute progressive stroke with middle cerebral artery occlusion caused by idiopathic hypereosinophilic syndrome: a case report.

BMC neurology
2021

Successful treatment of idiopathic hypereosinophilic syndrome with benralizumab in a pediatric patient.

The journal of allergy and clinical immunology. In practice
2020

The role of eosinophil morphology in distinguishing between reactive eosinophilia and eosinophilia as a feature of a myeloid neoplasm.

British journal of haematology
2020

Colon perforation due to cytomegalovirus infection in a patient with idiopathic hypereosinophilic syndrome: a case report.

BMC gastroenterology
2020

Idiopathic hypereosinophilic syndrome associated with rapid progression of cardiac, pulmonary and skin infiltration.

Cardiovascular journal of Africa
2020

[Diagnostic workup in front of an hypereosinophilia in 2020].

Annales de biologie clinique
2020

An unusual presentation of Idiopathic hypereosinophilic syndrome.

Journal of thrombosis and thrombolysis
2020

Idiopathic hypereosinophilic syndrome with formation of multiple liver mass lesions.

Clinical journal of gastroenterology
2020

Ascites in a young male: idiopathic FIP1L1-PDGFRA-negative hypereosinophilic syndrome.

JRSM open
2019

Idiopathic hypereosinophilic syndrome presenting as monoplegia.

BMJ case reports
2019

Idiopathic hypereosinophilic syndrome with cutaneous necrosis and multiorgan embolism.

JAAD case reports
2019

Hypereosinophilic dermatitis: generalised lichenification and gyrate erythema as the sole manifestation of idiopathic hypereosinophilic syndrome.

BMJ case reports
2020

Familial clustering of hypereosinophilic diseases treated with mepolizumab: a case report from Japan.

European annals of allergy and clinical immunology
2019

Loeffler Endocarditis in Idiopathic Hypereosinophilic Syndrome Demonstrated by Magnetic Resonance Imaging Effectively Treated by Corticosteroids.

Acta Cardiologica Sinica
2019

Acute kidney injury secondary to thrombotic microangiopathy associated with idiopathic hypereosinophilic syndrome: a case report and review of the literature.

Journal of medical case reports
2019

Skin-limited idiopathic hypereosinophilic syndrome presenting with retiform purpura.

JAAD case reports
2019

World Health Organization-defined eosinophilic disorders: 2019 update on diagnosis, risk stratification, and management.

American journal of hematology
2019

Long-term Follow-up of Severe Eosinophilic Hepatitis: A Rare Presentation of Hypereosinophilic Syndrome.

Rambam Maimonides medical journal
2019

Teaching NeuroImages: Multifocal cerebral infarcts as a presentation of idiopathic hypereosinophilic syndrome.

Neurology
2018

A case report of Löffler endocarditis in idiopathic hypereosinophilic syndrome: recovery is possible.

European heart journal. Case reports
2019

Idiopathic Hypereosinophilic Syndrome in an Elderly Female: A Case Report.

The American journal of case reports
2019

Idiopathic hypereosinophilic syndrome presenting with multiple organ damage: A case report.

Medicine
2018

[Chronic myeloid leukemia presenting with marked eosinophilia].

[Rinsho ketsueki] The Japanese journal of clinical hematology
2018

Idiopathic hypereosinophilic syndrome with cutaneous involvement: a comparative review of 32 cases.

BMJ case reports
2018

Eyelid Swelling and Subconjunctival Infiltration as Ophthalmic Manifestations in a Child with Idiopathic Hypereosinophilic Syndrome.

Korean journal of ophthalmology : KJO
2018

Acute pulmonary embolism and deep vein thrombosis secondary to idiopathic hypereosinophilic syndrome.

Respiratory medicine case reports
2019

Churg-Strauss Syndrome Presenting as Acute Necrotizing Eosinophilic Myocarditis: Concise Review of the Literature.

Current hypertension reviews
2018

A multimodality work-up of patients with Hypereosinophilia.

American journal of hematology
2019

Idiopathic hypereosinophilic syndrome with pulmonary hypertension.

Pulmonary circulation
2018

Dysphonia and dyspnea in idiopathic hypereosinophilic syndrome treated with Mepolizumab.

Respiratory medicine case reports
2019

The Diagnostic Work-Up of Hypereosinophilia.

Pathobiology : journal of immunopathology, molecular and cellular biology
2017

[Hypereosinophilic syndrome associated with cardiac involvement].

The Pan African medical journal
2018

A Case of Pulmonary Hypertension Associated with Idiopathic Hypereosinophilic Syndrome.

International heart journal
2018

Fatal Outcome of Imatinib in a Patient with Idiopathic Hypereosinophilic Syndrome.

Case reports in oncological medicine
2018

A young female presenting with heart failure secondary to eosinophilic myocarditis: a case report and review of the literature.

BMC research notes
2018

Severe Multi-Organ Failure and Hypereosinophilia: When to Call It "Idiopathic"?

Journal of investigative medicine high impact case reports
2018

[Portal vein thrombosis associated with idiopathic hypereosinophilic syndrome].

[Rinsho ketsueki] The Japanese journal of clinical hematology
2018

[A case report of idiopathic hypereosinophilic syndrome with pulmonary embolism].

Zhonghua nei ke za zhi
2019

The right atrium in idiopathic hypereosinophilic syndrome : Insights from the 3D speckle tracking echocardiographic MAGYAR-Path Study.

Herz
2017

Eosinophilic Myocarditis.

The American journal of the medical sciences
2017

Idiopathic hypereosinophilia is clonal disorder? Clonality identified by targeted sequencing.

PloS one
2017

[Pediatric idiopathic hypereosinophilic syndrome with pulmonary embolism: a case report and review of literature].

Zhonghua er ke za zhi = Chinese journal of pediatrics
2017

World Health Organization-defined eosinophilic disorders: 2017 update on diagnosis, risk stratification, and management.

American journal of hematology
2017

Idiopathic hypereosinophilic syndrome with 20 years of diagnostic delay.

Revista brasileira de hematologia e hemoterapia
2017

Therapeutic effect of anti-IL-5 on eosinophilic myocarditis with large pericardial effusion.

BMJ case reports
2017

When a death apparently associated to sexual assault is instead a natural death due to idiopathic hypereosinophilic syndrome: The importance of gamma-hydroxybutyric acid analysis in vitreous humor.

Legal medicine (Tokyo, Japan)
2017

Bone marrow morphology is a strong discriminator between chronic eosinophilic leukemia, not otherwise specified and reactive idiopathic hypereosinophilic syndrome.

Haematologica
2017

Idiopathic hypereosinophilic syndrome manifesting with eosinophilic cholecystitis and recurrent gastroenteritis.

Annals of hematology
2017

Total artificial heart implantation for biventricular failure due to eosinophilic myocarditis.

Journal of artificial organs : the official journal of the Japanese Society for Artificial Organs
2017

Hypereosinophilic Syndrome After Liver Transplantation: A Case Report and a Review of the Literature.

Transplantation
2017

Rapid Change in Mental Status in a Patient with Hypereosinophilia.

Case reports in hematology
2017

ANCA-Negative Churg-Strauss Syndrome Presenting as Acute Multiple Cerebral Infarcts: A Case Report.

Journal of stroke and cerebrovascular diseases : the official journal of National Stroke Association
2016

Idiopathic hypereosinophilic syndrome resulting in distal aortic thromboembolism in a dog.

Journal of veterinary emergency and critical care (San Antonio, Tex. : 2001)
2016

Idiopathic hypereosinophilic syndrome presenting with severe vasculitis successfully treated with imatinib.

World journal of clinical cases
2016

[Clinical characteristics and long- term therapeutic effects of 60 patients with idiopathic hypereosinophilic syndrome in a single center].

Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi
2016

Hypereosinophilic Syndrome: Hacettepe Experience.

Journal of pediatric hematology/oncology
2016

Idiopathic hypereosinophilic syndrome presenting as angina pectoris: A case report.

International journal of cardiology
2016

The significance of early screening with echocardiography in eosinophilic granulomatosis with polyangiitis.

Journal of medical ultrasonics (2001)
2016

Idiopathic hypereosinophilic syndrome presenting with hepatitis and achalasia.

Clinical journal of gastroenterology
2016

Eosinophilic pleural effusion as a manifestation of idiopathic hypereosinophilic syndrome.

Archivos de bronconeumologia
2016

Targeted next-generation sequencing identifies a subset of idiopathic hypereosinophilic syndrome with features similar to chronic eosinophilic leukemia, not otherwise specified.

Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
2015

An Unusual Case of Loffler Endomyocarditis after Takotsubo Cardiomyopathy Induced by Deep Neck Infection.

Acta Cardiologica Sinica
2016

Cardiopulmonary and Gastrointestinal Manifestations of Eosinophil- associated Diseases and Idiopathic Hypereosinophilic Syndromes: Multimodality Imaging Approach.

Radiographics : a review publication of the Radiological Society of North America, Inc
2015

A Case of Idiopathic Hypereosinophilic Syndrome Causing Mitral Valve Papillary Muscle Rupture.

Case reports in pediatrics
2015

Whole-exome sequencing and genome-wide methylation analyses identify novel disease associated mutations and methylation patterns in idiopathic hypereosinophilic syndrome.

Oncotarget
2015

Endobronchial Involvement in Idiopathic Hypereosinophilic Syndrome.

Journal of bronchology &amp; interventional pulmonology
2015

World Health Organization-defined eosinophilic disorders: 2015 update on diagnosis, risk stratification, and management.

American journal of hematology
2016

Comparison of two leukocytapheresis protocols in a case of idiopathic hypereosinophilic syndrome.

Journal of clinical apheresis
2015

A Rare Case of Aortic Valve Thrombosis in Patient with Idiopathic Hypereosinophilic Syndrome.

Case reports in cardiology
2015

Tyrosine Kinase Inhibitors and Therapeutic Antibodies in Advanced Eosinophilic Disorders and Systemic Mastocytosis.

Current hematologic malignancy reports
2015

Reversible Severe Eosinophilic Endomyocardial Fibrosis During Pregnancy: A Case Report.

Medicine
2016

Hypereosinophilic syndrome: an indolent rash with a serious cardiac complication.

Clinical and experimental dermatology
2015

Management of Hypereosinophilic Syndromes.

Immunology and allergy clinics of North America
Ver todos os 419 no EuropePMC

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Doenças relacionadas

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Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Idiopathic Hypereosinophilic Syndrome Presenting as Acute Encephalopathy Secondary to Watershed Ischemic Stroke.
    Annals of Indian Academy of Neurology· 2026· PMID 41784094mais citado
  2. Idiopathic Hypereosinophilic Syndrome in a Child- Rare Disease with Fulminant Clinical Course.
    Indian journal of hematology &amp; blood transfusion : an official journal of Indian Society of Hematology and Blood Transfusion· 2026· PMID 41728149mais citado
  3. Laboratory Aspects and Diagnostic Challenges in a Case of Hypereosinophilic Syndrome.
    Cureus· 2026· PMID 41700233mais citado
  4. Real-world outcomes after anti-IL-5/anti-IL-5R&#x3b1; treatment for hypereosinophilic syndrome: Systematic literature review.
    The journal of allergy and clinical immunology. Global· 2026· PMID 41488420mais citado
  5. Clinical Utility of 18 F-FDG PET/CT in Patients With Hypereosinophilia : An 11-year Single-center Experience.
    Clinical nuclear medicine· 2026· PMID 41307472mais citado
  6. Coronary vasospasm in a patient with RUNX1 mutation in eosinophilic leukemia and hypereosinophilic syndrome: A case report.
    J Cardiol Cases· 2026· PMID 41994071recente
  7. Idiopathic Hypereosinophilic Syndrome in a Patient with End-Stage Renal Disease.
    Clin Lab· 2026· PMID 41945721recente
  8. Aortic Valve Annular Parameters in Idiopathic Hypereosinophilic Syndrome-A Detailed Investigation from the Three-Dimensional Speckle-Tracking Echocardiographic MAGYAR-Path Study.
    Biomedicines· 2026· PMID 41898261recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:3260(Orphanet)
  2. OMIM OMIM:607685(OMIM)
  3. MONDO:0011895(MONDO)
  4. GARD:16625(GARD (NIH))
  5. Variantes catalogadas(ClinVar)
  6. Busca completa no PubMed(PubMed)
  7. Artigo Wikipedia(Wikipedia)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Síndrome hipereosinofílico idiopático
Compêndio · Raras BR

Síndrome hipereosinofílico idiopático

ORPHA:3260 · MONDO:0011895
Prevalência
Unknown
Herança
Not applicable
CID-10
D47.5 · Outras neoplasias de comportamento incerto ou desconhecido dos tecidos linfático, hematopoético e tecidos correlatos
CID-11
Ensaios
1 ativos
Início
Adult
Prevalência
0.0 (Europe)
MedGen
UMLS
C0206141
EuropePMC
Wikipedia
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