A Síndrome Hipereosinofílica (HES) é um grupo de doenças raras e variadas. Ela é caracterizada por um aumento constante e significativo de um tipo de glóbulo branco chamado eosinófilo no sangue, e/ou pelo acúmulo desses eosinófilos nos tecidos e órgãos do corpo. Isso tudo está associado a uma grande variedade de sintomas que refletem os danos que os próprios eosinófilos causam a esses tecidos e órgãos.
Introdução
O que você precisa saber de cara
A Síndrome Hipereosinofílica (HES) é um grupo de doenças raras e variadas. Ela é caracterizada por um aumento constante e significativo de um tipo de glóbulo branco chamado eosinófilo no sangue, e/ou pelo acúmulo desses eosinófilos nos tecidos e órgãos do corpo. Isso tudo está associado a uma grande variedade de sintomas que refletem os danos que os próprios eosinófilos causam a esses tecidos e órgãos.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
Encontrou um erro ou informação desatualizada? Sugira uma correção →
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
+ 34 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 88 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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
5 genes identificados com associação a esta condição. Padrão de herança: Not applicable, Unknown.
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
Cell membraneCell projection, ciliumGolgi apparatus
Tyrosine-protein kinase that acts as a cell-surface receptor for fibroblast growth factors and plays an essential role in the regulation of embryonic development, cell proliferation, differentiation and migration. Required for normal mesoderm patterning and correct axial organization during embryonic development, normal skeletogenesis and normal development of the gonadotropin-releasing hormone (GnRH) neuronal system. Phosphorylates PLCG1, FRS2, GAB1 and SHB. Ligand binding leads to the activati
Cell membraneNucleusCytoplasm, cytosolCytoplasmic vesicle
Pfeiffer syndrome
A syndrome characterized by the association of craniosynostosis, broad and deviated thumbs and big toes, and partial syndactyly of the fingers and toes. Three subtypes are known: mild autosomal dominant form (type 1); cloverleaf skull, elbow ankylosis, early death, sporadic (type 2); craniosynostosis, early demise, sporadic (type 3).
Transcriptional repressor; binds to the DNA sequence 5'-CCGGAAGT-3'. Plays a role in hematopoiesis and malignant transformation
Nucleus
Tyrosine-protein kinase that acts as a cell-surface receptor for homodimeric PDGFB and PDGFD and for heterodimers formed by PDGFA and PDGFB, and plays an essential role in the regulation of embryonic development, cell proliferation, survival, differentiation, chemotaxis and migration. Plays an essential role in blood vessel development by promoting proliferation, migration and recruitment of pericytes and smooth muscle cells to endothelial cells. Plays a role in the migration of vascular smooth
Cell membraneCytoplasmic vesicleLysosome lumen
Component of the cleavage and polyadenylation specificity factor (CPSF) complex that plays a key role in pre-mRNA 3'-end formation, recognizing the AAUAAA signal sequence and interacting with poly(A) polymerase and other factors to bring about cleavage and poly(A) addition. FIP1L1 contributes to poly(A) site recognition and stimulates poly(A) addition. Binds to U-rich RNA sequence elements surrounding the poly(A) site. May act to tether poly(A) polymerase to the CPSF complex
Nucleus
Medicamentos aprovados (FDA)
1 medicamento encontrado nos registros da FDA americana.
Variantes genéticas (ClinVar)
852 variantes patogênicas registradas no ClinVar.
Classificação de variantes (ClinVar)
Distribuição de 171 variantes classificadas pelo ClinVar.
Vias biológicas (Reactome)
40 vias biológicas associadas aos genes desta condição.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Síndromes hipereosinofílicos
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
Ensaios em destaque
🟢 Recrutando agora
6 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.
Outros ensaios clínicos
139 ensaios clínicos encontrados, 10 ativos.
Publicações mais relevantes
Mostrando amostra de 200 publicações de um total de 934
Long-term outcomes of autologous cell transplantation for patients with non-TAO autoimmune disease-related critical limb ischemia.
Autoimmune disease-related critical limb ischemia (AD-CLI) represents a significant portion of no-option critical limb ischemia (NO-CLI). Despite the demonstrated safety and efficacy of cell transplantation for NO-CLI, most studies focused on thromboangiitis obliterans (TAO). There remains a scarcity of reports on cell transplantation for AD-CLI induced by systemic lupus erythematosus (SLE), hypereosinophilic syndrome (HES), and so on. From May 2011 to May 2024, 22 patients with 36 ischemic limbs were enrolled. The primary outcome was amputation, whereas secondary outcomes included toe-brachial index (TBI), Wong-Baker Faces Pain Rating Scale (WBFPS), transcutaneous oxygen pressure (TcPO2), and so on. Among them, 8 had SLE, 11 had HES, and 3 had eosinophilic granulomatous polyangiitis (EGPA). During the follow-up, one EGPA patient died, two patients underwent major amputation, and five underwent minor amputation. The 2-year major and total amputation-free survival rates were 86.4% and 72.7%, respectively. Critical limb ischemia (CLI) relief was observed in 14 patients, with a 1-year cumulative CLI-free survival rate of 61.0%. Significant improvements were noted in postoperative TBI and WBFPS. Autologous cell transplantation showed satisfactory safety and efficacy outcomes for non-TAO AD-CLI patients. Validation of the conclusions awaits more evidence based on the long-term outcomes of a larger number of patients.
Natural History of Patients With Histologically Proven Acute Eosinophilic Myocarditis.
No large registries of patients with acute eosinophilic myocarditis (EM) are available. However, EM is perceived as a cardiac disease with high mortality, affecting mainly young and middle-aged adults according to small series and case reports. Awareness of the clinical presentation, associated systemic conditions, treatments, and outcomes of this uncommon condition is an unmet need. In this international, multicenter, retrospective cohort study, 53 centers screened 193 patients with histologically proven acute EM between 1992 and 2023. After the exclusion of patients with insufficient data (n=10), symptoms lasting >30 days (n=19), or histological diagnosis not confirmed after review (n=8), 156 patients were included. Median age at presentation was 48 years (first to third quartile, 34-59 years) with male predominance (67.3%), and only 2 were pediatric cases (≤16 years of age; 1.3%). The main signs and symptoms at presentation were dyspnea (75.6%), fever (61.3%), and chest pain (53.2%). Unexpectedly, peripheral eosinophilia was reported in only 57.4% of cases, with a median cell count of 630 eosinophils/μL. The median left ventricular ejection fraction at presentation was 32% (first to third quartile, 25%-48%). The disorders most frequently associated with EM were eosinophilic granulomatosis with polyangiitis (22.4% of cases) and hypersensitivity forms (14.1%). Idiopathic/undefined forms accounted for 44.9% of cases, and miscellaneous causes accounted for 18.6%. In-hospital death or need for heart transplantation (HTx) occurred in 23 patients (14.7%; 22 deaths and 1 HTx), despite 43.6% being treated with temporary mechanical circulatory support and 92.9% being treated with immunosuppressive agents. Estimated rates of death or HTx at 1 and 3 years were 19.0% and 23.8%. Increased age, decreased left ventricular ejection fraction on admission, and no immunosuppressive therapy during hospitalization were independent predictors of death or HTx. A nonsignificant higher occurrence of deaths or HTx was observed in the hypersensitivity form (46.1%) compared with the eosinophilic granulomatosis with polyangiitis-associated form (13.1%) at 3 years (P=0.15). Acute EM can often present without peripheral eosinophilia, and rates of in-hospital and midterm mortality or HTx are high. Endomyocardial biopsy is required to reach the final diagnosis of EM because relying on peripheral eosinophilia can lead to missing diagnosis. In-hospital immunosuppression is associated with HTx-free survival, although tailored immunosuppressive therapies are needed to improve outcomes. URL: https://www.clinicaltrials.gov; Unique identifier: NCT06447935.
Idiopathic Hypereosinophilic Syndrome Presenting as Acute Encephalopathy Secondary to Watershed Ischemic Stroke.
Idiopathic Hypereosinophilic Syndrome in a Child- Rare Disease with Fulminant Clinical Course.
Hypereosinophilic syndrome presenting as pleural and pericardial effusion in a Pediatric patient: a rare case report.
Hypereosinophilic syndrome is a rare disorder in children, particularly when presenting with simultaneous pleural and pericardial effusions. We report the case of a 4-year-old girl who presented with fever, cough, shortness of breath, and chest pain. Clinical evaluation revealed bilateral pleural effusion and later pericardial effusion with impending tamponade. Laboratory investigations showed marked hypereosinophilia, while infectious, rheumatologic, and malignant causes were excluded. Bone marrow findings confirmed eosinophilic predominance without atypia. The patient underwent pericardiocentesis and was treated with corticosteroids, resulting in clinical improvement and normalization of eosinophil counts. On follow-up, she remained asymptomatic with resolution of effusions. This case underscores the importance of considering HES as a differential diagnosis in pediatric patients with eosinophilic effusions and highlights the role of early recognition and corticosteroid therapy in preventing life-threatening complications.
Publicações recentes
Coronary vasospasm in a patient with RUNX1 mutation in eosinophilic leukemia and hypereosinophilic syndrome: A case report.
Case Report: Hypereosinophilic syndrome misdiagnosed as atopic dermatitis due to refractory pruritic rash masking peripheral neuropathy.
Idiopathic Hypereosinophilic Syndrome in a Patient with End-Stage Renal Disease.
Benralizumab versus placebo for hypereosinophilic syndrome: a randomized, placebo-controlled phase 3 trial.
Eosinophilic Organ Complications Associated with Dupilumab Therapy - Narrative Review and Current Evidence.
📚 EuropePMC1.556 artigos no totalmostrando 197
Hypereosinophilic syndrome presenting as pleural and pericardial effusion in a Pediatric patient: a rare case report.
Oxford medical case reportsPossible Involvement of Eosinophil ETosis in Hepatic Injury Associated with Hypereosinophilic Syndrome.
Modern rheumatology case reportsRedefining Hematology Referral Thresholds for Eosinophilia.
European journal of haematologyMolecular Pathogenesis and Targeted Therapies for Myeloproliferative Hypereosinophilic Neoplasms.
Current hematologic malignancy reportsBiologics Use in Eosinophilic Lung Disease: Controversies and Consensus.
The journal of allergy and clinical immunology. In practiceDifferential Diagnosis of Eosinophilic Lung Diseases.
The journal of allergy and clinical immunology. In practiceIdiopathic Hypereosinophilic Syndrome Presenting as Acute Encephalopathy Secondary to Watershed Ischemic Stroke.
Annals of Indian Academy of NeurologyLong-term outcomes of autologous cell transplantation for patients with non-TAO autoimmune disease-related critical limb ischemia.
Cell transplantationLoss of NF-κB-inducing kinase (NIK) affects eosinophilopoiesis in a murine model of Hypereosinophilic syndrome.
Journal of leukocyte biologyAcute hemiplegia as initial presentation in FIP1L1-PDGFRA-rearranged myeloid neoplasm with eosinophilia: a case report.
Frontiers in oncology[Incidental finding of negative T waves: a case of endomyocardial fibrosis].
Giornale italiano di cardiologia (2006)Long Term Outcomes in Patients With Non-Infectious Mixed Cryoglobulinemic Vasculitis.
American journal of hematologyIdiopathic Hypereosinophilic Syndrome in a Child- Rare Disease with Fulminant Clinical Course.
Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood TransfusionCase Report of a Lymphocytic Variant Hypereosinophilic Syndrome Mimicking T-Cell Lymphoma: A Diagnostic Challenge and Therapeutic Response to Mepolizumab.
The Australasian journal of dermatologyLaboratory Aspects and Diagnostic Challenges in a Case of Hypereosinophilic Syndrome.
CureusIschemic stroke complicated by intracerebral hemorrhage in patients with hypereosinophilic syndrome: a case series.
Therapeutic advances in neurological disordersIL-5 CAR-T cell therapy induces effective remission in hypereosinophilic disorders.
Journal of hematology & oncologyNovel Diagnostic Approaches for Eosinophilic Lung Diseases.
The journal of allergy and clinical immunology. In practiceNatural History of Patients With Histologically Proven Acute Eosinophilic Myocarditis.
CirculationCatastrophic antiphospholipid syndrome with eosinophilia mimicking hypereosinophilic syndromes with disseminated intravascular coagulation: a case report.
Journal of medical case reportsFatal Eosinophilic Pulmonary Vasculitis in a Neonate - The First Documented Case in the English Literature.
Annals of clinical and laboratory scienceCase Report: Triple autoimmune overlap: rheumatoid arthritis, systemic lupus erythematosus, and hypereosinophilic asthma with systemic manifestations.
Frontiers in immunologyFrom hypereosinophilia to hypereosinophilic syndrome: real-world application of a two-tailed approach for HES diagnosis.
Frontiers in immunologyFulminant Idiopathic Hypereosinophilic Syndrome Presenting with Cardiac, Neurologic and Haematologic Involvement.
European journal of case reports in internal medicineSevere mitral regurgitation in the course of hypereosinophilic syndrome.
Polish archives of internal medicineThe value of left ventricular and left atrial strains in eosinophilic myocarditis and hypereosinophilic syndrome.
Cardiovascular diagnosis and therapyReal-world outcomes after anti-IL-5/anti-IL-5Rα treatment for hypereosinophilic syndrome: Systematic literature review.
The journal of allergy and clinical immunology. GlobalGenetic mutations in lymphocytic variant of hypereosinophilic syndrome: study of five siblings.
Frontiers in medicineEvaluation and differential diagnosis of eosinophilia: A tertiary allergy center experience.
Tuberkuloz ve toraksA Rapid Drug-Induced Granulomatous Dermatitis to Amlodipine.
The American journal of cardiologyBenralizumab: Bringing winds of change to eosinophil-associated diseases.
The World Allergy Organization journalANCA-Negative Eosinophilic Granulomatosis With Polyangiitis Presenting With Duodenal Stenosis: A Case Report.
The American journal of case reportsRapid Improvement of Refractory Cutaneous Hypereosinophilic Syndrome with Abrocitinib Plus Methylprednisolone: A Case Report.
Clinical, cosmetic and investigational dermatologyHypereosinophilia: Clinical Presentations and Diagnostic Distinction between Syndromic and Reactive Entities - A Case Series.
Romanian journal of internal medicine = Revue roumaine de medecine interneRefractory erythroderma in idiopathic hypereosinophilic syndrome: A case treated with mepolizumab and abrocitinib.
SAGE open medical case reportsFrom bone marrow to the heart: a case report of cardiac involvement in myeloproliferative-associated hypereosinophilic syndrome.
Future cardiologyLoffler endocarditis as an initial manifestation of lung adenocarcinoma: A case report.
MedicineAcute multisystemic thromboembolism manifesting as cerebral infarction, myocardial injury, and quadriparesis: a novel triad of idiopathic hypereosinophilic syndrome.
Frontiers in medicineToward Better Patient-Centered Care of Hypereosinophilic Syndrome Using Novel Patient-Reported Outcome Measure Tool.
The journal of allergy and clinical immunology. In practiceSteroid resistant, osimertinib induced hypereosinophilic syndrome (HES) treated with Benralizumab in a patient with metastatic adenocarcinoma of lung.
Respiratory medicine case reportsBiologic Therapy for Chronic Rhinosinusitis with Nasal Polyps: Case Analysis and Proposal of a Qualification Scheme.
Otolaryngologia polska = The Polish otolaryngologyLymphocytic Hypereosinophilic Syndrome Evolving to Fatal Hemophagocytic Lymphohistiocytosis: Cytokine Signatures.
Clinical laboratoryIschemic Stroke in Hypereosinophilic Syndrome: A Clinicopathologic Study of Two Cases.
Neuropathology : official journal of the Japanese Society of NeuropathologyMepolizumab for hypereosinophilic syndrome: effectiveness and safety from real-world evidence.
Frontiers in immunologyPrediction model to identify patients with hypereosinophilic syndrome using real-world data.
The journal of allergy and clinical immunology. GlobalHypereosinophilic syndrome with pulmonary and hepatic involvement mimicking autoimmune disease.
Oxford medical case reportsClinical Utility of 18 F-FDG PET/CT in Patients With Hypereosinophilia : An 11-year Single-center Experience.
Clinical nuclear medicineEndomyocardial Fibrosis Associated With Hypereosinophilic Syndrome: Diagnostic and Management Insights From a Case Report.
Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & InterventionsMepolizumab improves health-related quality of life in patients with hypereosinophilic syndrome.
European journal of internal medicineThe Clinical Burden of Hypereosinophilic Syndrome in a Large United States Cohort.
The journal of allergy and clinical immunology. In practiceLoeffler endocarditis in hypereosinophilic syndrome, the paradox of absent peripheral eosinophilia: a case report.
European heart journal. Case reportsIdiopathic 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 surgeryNon-atherosclerotic myocardial infarction in hypereosinophilic syndrome: emerging insights and therapeutic approaches.
Heart (British Cardiac Society)Cellular profiling identifies targetable T cell phenotypes in lymphocytic variant hypereosinophilic syndrome.
The Journal of clinical investigationParasitic-induced Loeffler's syndrome: A systematic review based on clinical reports.
Journal of infection and public health[Clinical analysis of six eosinophilia cases with Löeffler endocarditis as the main clinical feature].
Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhiIdiopathic hypereosinophilic syndrome associated with multivessel coronary artery ectasia: a case report.
The Pan African medical journalPanuveitis Associated With Idiopathic Hypereosinophilic Syndrome.
Journal of vitreoretinal diseasesEosinophilic Cardiac Involvement.
The journal of allergy and clinical immunology. In practiceEosinophilic Myocarditis Treated with IL-5 Blockade: An Integrated Case Report and Literature Review.
Journal of clinical medicinePediatric Severe Eosinophilia: Etiological Spectrum, Diagnostic Algorithm, and Case-Based Insights From a Tertiary Care Center.
International journal of laboratory hematologyIdiopathic hypereosinophilic syndrome: Potential pathologic somatic gene variants identified by exome sequencing.
The journal of allergy and clinical immunology. GlobalThe role of eosinophils and their activation state in hypereosinophilia-associated heart disease.
Frontiers in immunologyHypereosinophilic Syndrome Presenting as Coronary Artery Spasm and ST-Segment Elevation Myocardial Infarction.
JACC. Case reportsIdiopathic hypereosinophilic syndrome in a female Sprague Dawley rat.
Laboratory animalsOrgan-Restricted Hypereosinophilic Syndrome: A Case of Recurrent Hypereosinophilic Myopericarditis.
JACC. Case reportsDevelopment and Validation of the FIP Score for the Screening of FIP1L1::PDGFRA-Associated Hypereosinophilic Syndrome.
The journal of allergy and clinical immunology. In practiceDevelopment of a Patient-Reported Outcome Measure for Hypereosinophilic Syndrome.
The journal of allergy and clinical immunology. In practiceThe hypereosinophilia dilemma: what's beyond cardiac involvement?
Romanian journal of internal medicine = Revue roumaine de medecine interneA Clinical Conundrum in Patients With a Common Clinical Spectrum but Rare Differentials: Hyper-Immunoglobulin E Syndrome and Idiopathic Eosinophilia.
CureusEosinophilic ascites and eosinophilic gastrointestinal diseases.
European annals of allergy and clinical immunologyParadoxical Use of Benralizumab in Reactive Hypereosinophilia from Toxocariasis and Tuberculosis Co-Infection-Case Report and Literature Review.
International journal of molecular sciencesIdiopathic Hypereosinophilic Syndrome Presenting As Eosinophilic Ascites and Pleural Effusion in an Elderly Male: A Rare Clinical Manifestation.
Cureus[Biologicals in Type-2-Inflammation - from Chronic Rhinosinusitis with Nasal Polyps to Hypereosinophilic Syndrome].
Laryngo- rhino- otologieBenralizumab in a Patient With Refractory Eosinophilic Endocarditis.
Journal of medical casesA case report of fulminant eosinophilic myo-pericarditis related to checkpoint inhibitors complicated by acute heart failure and cardiac tamponade.
European heart journal. Case reports[Efficacy and survival outcomes of patients with lymphocytic variant hypereosinophilic syndrome].
Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhiEosinophilic lung disease with a tumour lysis-like syndrome.
BMJ case reportsCase Report: Idiopathic hypereosinophilic syndrome presenting with gastrointestinal involvement mimicking IBD: a diagnostic challenge.
Frontiers in medicine[A Case of Idiopathic Hypereosinophilic Syndrome Treated with Mepolizumab].
Acta medica portuguesaHypereosinophilic Syndrome in a Patient With Cystic Fibrosis: A Rare Case of Cardiac Involvement and Response to Mepolizumab.
CureusDevelopment and implementation of Phleos, a web-based tool for the data collection on Hypereosinophilic syndrome: the Italian Network on HES (INHES) study protocol.
Frontiers in immunologyA Rare Presentation of Bilateral Foot Drop in Anti-neutrophil Cytoplasmic Antibody (ANCA)-Negative Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss Syndrome): A Diagnostic Challenge.
CureusHypereosinophilic syndrome with elevated IgG4 and T-cell clonality: A report of two cases.
Open life sciencesDiagnostic and therapeutic challenges in colonic eosinophilic abscess complicated with eosinophilic vasculitis: a case report and literature review.
Frontiers in medicineClinical and immunological biomarkers in hypereosinophilic syndrome: the second step after diagnostic algorithms.
Frontiers in medicineAn Uncommon Case of Non-Specific Interstitial Pneumonia (NSIP) Associated With Idiopathic Hypereosinophilic Syndrome (HES) Reversed by Mepolizumab.
Respirology case reportsPrimary care physicians play a crucial role in diagnosing and managing rare eosinophilic diseases: HES and EGPA.
Frontiers in medicineDiagnosis of Loeffler Endocarditis in a Patient with a Cerebrovascular Event.
Turk Kardiyoloji Dernegi arsivi : Turk Kardiyoloji Derneginin yayin organidirLymphocytic Variant Hypereosinophilic Syndrome With Extensive Mucocutaneous Involvement Successfully Treated With Ruxolitinib.
American journal of hematologyHypereosinophilic syndrome presented with portal, superior mesenteric, and splenic vein thromboses in a child.
Pediatrics international : official journal of the Japan Pediatric SocietyHypereosinophilic Syndrome With Sialadenitis and Orbital Inflammation: A Case Report.
Journal of investigational allergology & clinical immunologyHigh Disease Burden and Oral Corticosteroid Use in Patients with Hypereosinophilic Syndrome and Eosinophilic Granulomatosis with Polyangiitis: Country-Level Insights into Real-World Management in Europe.
Journal of clinical medicineOptimizing diagnostic and management pathways for patients with eosinophilia of unknown origin: a multidisciplinary protocol for urgent and non-urgent evaluation.
Frontiers in medicineReversible Dementia With Hypereosinophilia: An Intricate Connection.
CureusTh2-High Severe Asthma with Hypereosinophilia in the Spectrum of Type 2 Inflammatory Diseases.
International journal of molecular sciencesSteroid-sparing benefits of biologic use in hypereosinophilic syndrome and substantial disease burden across subtypes.
Frontiers in allergyCoronary Artery Spasm as a Rare Manifestation of Hypereosinophilic Syndrome: A Case Report.
Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & InterventionsHypereosinophilic Syndrome Presenting with Digestive Symptoms in Pediatric Patients: A Portuguese Tertiary Hospital Case Series.
GE Portuguese journal of gastroenterologyFour challenging cases of eosinophilic endocarditis or myocarditis with literature review.
Journal of cardiothoracic surgeryTracking Public Interest in Rare Diseases and Eosinophilic Disorders in Germany: Web Search Analysis.
JMIR infodemiologyHypereosinophilia in Children.
Current allergy and asthma reportsLoeffler Endocarditis with Antineutrophil Cytoplasmic Antibody-associated Vasculitis after COVID-19 Vaccination.
Internal medicine (Tokyo, Japan)Hypereosinophilia: clinical and therapeutic approach in 2025.
Current opinion in allergy and clinical immunologyLoeffler endocarditis revealing chronic eosinophilic leukaemia with FIP1L1-PDGFRA rearrangement: a case report.
European heart journal. Case reportsBenralizumab for acute thromboembolism in hypereosinophilic syndrome: a case report.
Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical ImmunologyEosinophilia and gastrointestinal symptoms in Immunosuppression: a classic but overlooked diagnosis.
Revista espanola de enfermedades digestivasExpansion of multiple CD4+ T-cell lineages in lymphocytic variant hypereosinophilic syndrome.
The Journal of allergy and clinical immunologyComplete hematological response with imatinib mesylate in a corticosteroid refractory idiopathic hypereosinophilic syndrome in person living with HIV: A case report.
International journal of STD & AIDSEosinophilic complications during dupilumab therapy for type 2 diseases: a systematic review.
RhinologyImproving patient outcomes: Mepolizumab's impact in IL-5-mediated diseases.
Lung India : official organ of Indian Chest SocietyBiologic Agents in Idiopathic Hypereosinophilic Syndrome.
Pharmaceuticals (Basel, Switzerland)A newly identified pathology of episodic angioedema with hypereosinophilia (Gleich syndrome) revealed by nultiomics analysis.
The journal of allergy and clinical immunology. GlobalUnexpected hypereosinophilia after Sinopharm vaccination: a case report.
BMC infectious diseasesInflammation and INOCA: Can Fat Attenuation Indexing by Coronary CT Angiography Help Identify Coronary Inflammation?
JACC. Case reportsHypereosinophilic syndrome with Löffler endocarditis in a patient with Rhupus syndrome.
Clinical and experimental rheumatologyWhen Eosinophils Speak: A Case of Strongyloides stercoralis Infection.
Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood TransfusionInsights from comparison of serum IgG4 between T2-eosinophilic asthma and eosinophilic granulomatosis with polyangiitis/idiopathic hypereosinophilic syndrome.
Clinical and experimental rheumatology11-Year-Old Boy With B-ALL-Induced Hypereosinophilic Syndrome Presenting as Acute Encephalopathy.
PediatricsLymphocytic Variant Hypereosinophilic Syndrome: Case Series From a Tertiary Referral Center in Canada.
EJHaemEosinophilic granulomatosis with polyangiitis: Patient profiles from a large US allergy practice.
The journal of allergy and clinical immunology. GlobalLong-Term Efficacy and Safety of Benralizumab Treatment for PDGFRA-Negative Hypereosinophilic Syndrome.
The journal of allergy and clinical immunology. In practiceClonal eosinophilia with exclusive pulmonary involvement driven by PDGFRA rearrangement treated with imatinib: A case report.
Leukemia research reportsHighlights from the plenary session: eosinophilic granulomatosis with polyangiitis and hypereosinophilic syndrome.
Rheumatology (Oxford, England)Hypereosinophilic Syndrome in a Patient With Complex Pulmonary Hypertension and Sjogren's Syndrome: A Case Report.
CureusMore Than Just Stool: Hypereosinophilic Syndrome Presenting as Persistent Diarrhea in a Patient With Ulcerative Colitis.
Journal of Brown hospital medicineEfficacy of anti-interleukin 5 therapy in hypereosinophilic syndrome: An updated systematic review and meta-analysis.
Allergy and asthma proceedingsClinical Management of Persistent Hypereosinophilia.
European journal of haematologyT-cell lymphoproliferative disorder with a STAT3 mutation causing a lymphocytic variant of hypereosinophilic-like syndrome without eosinophilia.
Journal of hematopathologyDouble Trouble: Coronary Aneurysm and Löffler Endocarditis in Hypereosinophilic Syndrome.
Circulation. Heart failureRefractory Idiopathic Hypereosinophilic Syndrome Presenting with Myocarditis and Responding to Imatinib: A Case Report.
Saudi journal of medicine & medical sciencesNovel ETV6::RAPGEF6 fusion gene in chronic eosinophilic leukemia: compiling evidence on the role of IL3 overexpression in tumorigenesis.
Annals of hematology68 Ga-FAPI PET/CT in a Patient With Multiple Aneurysms Due to Idiopathic Hypereosinophilic Syndrome.
Clinical nuclear medicineNF-κB-inducing kinase (NIK): an emerging therapeutic target in human disease.
Expert opinion on therapeutic targetsThrombotic prosthetic valve dysfunction requiring valve re-replacement after mitral valve replacement for caseous calcification with hypereosinophilic syndrome: a case report.
European heart journal. Case reportsEosinophils: old cells, new directions.
Frontiers in medicineIdiopathic Hypereosinophilic Syndrome With Cutaneous Manifestations: A Case Report.
CureusPulmonary Arterial Hypertension Associated With Idiopathic Hypereosinophilic Syndrome: Importance of Eosinophilia Control With Steroid Therapy.
CJC openMyocarditis with neurological and dermatological involvement in idiopathic Hypereosinophilic syndrome: Case report.
Radiology case reportsWells' Syndrome Associated With Idiopathic Hypereosinophilic Syndrome in a Child: A Case Report.
CureusSingle-center off-label benralizumab use for refractory hypereosinophilic syndrome demonstrates satisfactory safety and efficacy.
EJHaemEosinophilic Myocarditis: A Concise Review.
Current cardiology reportsCryptic to conventional cytogenetics: Philadelphia-like ALL presenting with hypereosinophilia.
BMJ case reportsA real-world study to evaluate effectiveness of mepolizumab in treating severe asthma in Taiwan (REMIT).
Therapeutic advances in respiratory diseaseHypereosinophilic Syndrome Complicated by Eosinophilic Myocarditis: Embolic Stroke or Eosinophilic Stroke? A Case Report.
Turk Kardiyoloji Dernegi arsivi : Turk Kardiyoloji Derneginin yayin organidirThe Cleveland Clinic experience of eosinophilic myocarditis in the setting of hypereosinophilic syndrome: demographics, cardiac imaging, and outcomes.
Cardiovascular diagnosis and therapyMepolizumab in patients with lymphoid-variant hypereosinophilic syndrome: A multicenter prospective study.
The Journal of allergy and clinical immunologyA Rare Case of Eosinophilic Myocarditis Described With 18F-FDG PET/CT.
Clinical nuclear medicineAcute Border Zone Infarcts in Hypereosinophilic Syndrome.
Kansas journal of medicineHypereosinophilia and Hypereosinophilic Syndromes: First Findings From a Nationwide Multicenter Cohort.
AllergyCase Report: Loeffler endocarditis as a cause of left ventricular thrombosis in young women: a case study and literature review.
Frontiers in cardiovascular medicineVectorized Human Antibody-Mediated Anti-Eosinophil Gene Therapy.
Human gene therapy[The evaluation of a patient with eosinophilia].
Nederlands tijdschrift voor geneeskundeFulminant eosinophilic myocarditis and refractory ventricular arrhythmias requiring mechanical circulatory support: a case report.
European heart journal. Case reportsHyper eosinophilic diseases - diagnosis and therapeutic approach - practical position of the Polish working group.
Otolaryngologia polska = The Polish otolaryngologyCardiac Abnormalities in Hypereosinophilic Syndromes.
Arquivos brasileiros de cardiologiaChronic eosinophilic leukaemia-Not otherwise specified: Clinical features, genomic insight and therapeutic strategies.
British journal of haematologyA Storm of Polyserositis: Unravelling Multisystem Effusions in Hypereosinophilic Syndrome.
CureusManagement of multiple valve replacement operations for a young patient with hypereosinophilic syndrome and valvular heart disease: a case report.
General thoracic and cardiovascular surgery casesUnexpected Multiple Gastrointestinal Cancers in a Patient with Chronic Eosinophilia: A Case Report.
Journal of gastrointestinal cancerDupilumab Use in Patients With Hypereosinophilic Syndromes: A Multicenter Case Series and Review of the Literature.
The journal of allergy and clinical immunology. In practice[Hypereosinophilic syndrome, case report and diagnostic approach].
Revista medica del Instituto Mexicano del Seguro SocialNon-episodic angioedema with eosinophilia as a differential diagnosis of eosinophilia in young females.
The World Allergy Organization journalMultiple Cerebral Infarcts and Encephalopathy as the First Clinical Manifestations of Hypereosinophilic Syndrome: A Case Report and Narrative Review.
Pulse (Basel, Switzerland)Imatinib is effective in some PDGFRA/B-negative hypereosinophilic syndromes: A step closer to unveiling underlying mechanisms.
British journal of haematologyAGA Clinical Practice Update on Esophageal Dysfunction Due to Disordered Immunity and Infection: Expert Review.
Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological AssociationMepolizumab-responsive recurrent eosinophilic eruption without peripheral eosinophilia.
JAAD case reportsA rare case of PDGFRA-positive chronic eosinophilic leukemia.
Indian journal of pathology & microbiologyAn Uncommon Cause of Acute Heart Failure: A Case Report.
CureusEosinophilic myocarditis: a diagnostic challenge and treatment dilemma-a case report.
European heart journal. Case reportsPhase II trial of imatinib mesylate in patients with PDGFRA/B-negative hypereosinophilic syndrome.
British journal of haematologyBiologics in Hypereosinophilic Syndrome and Eosinophilic Granulomatosis with Polyangiitis.
Immunology and allergy clinics of North AmericaRapid identification of primary atopic disorders (PAD) by a clinical landmark-guided, upfront use of genomic sequencing.
Allergologie selectSevere Acute Respiratory Distress in a Child with Hypereosinophilic Syndrome: A Case Report.
JNMA; journal of the Nepal Medical AssociationA hybrid case of eosinophilic folliculitis and eosinophilic cellulitis associated with hypereosinophilic syndrome.
Skin health and diseaseA Challenging Case of Idiopathic Hypereosinophilic Syndrome: An Itching in the Brain.
CureusIncessant Ventricular Tachycardia: An Atypical Presentation of Chronic Eosinophilic Leukemia.
JACC. Case reportsPossible Association of CD3+CD4-CD8- Phenotype of T-Cell Lymphoma with Peripheral Blood Eosinophilia.
International archives of allergy and immunologyBeyond the norm: Illuminating eosinophilic ascites with 2 rare and intriguing cases.
Radiology case reportsSuccessful novel use of dupilumab for gastrointestinal involvement of idiopathic hypereosinophilic syndrome: case report and review of the literature.
Clinical journal of gastroenterologyProbable idiopathic hypereosinophilic syndrome: A case report of severe multi-organ eosinophilic involvement in a young male presenting with heart failure.
SAGE open medical case reportsChronic cough, wheezing, and atelectasis following COVID-19: A case report on the diagnostic evaluation and effective treatment of a life-threatening asthma mimicker.
Pediatric pulmonologyIdiopathic hypereosinophilic syndromes and rare dysimmune conditions associated with hyper-eosinophilia in practice: An innovative multidisciplinary approach.
The World Allergy Organization journalDiagnosis of rare Loffler endocarditis by cardiac ultrasound: a case report and literature review.
Medical ultrasonographyEndomyocardial involvement in asymptomatic Latin American migrants with eosinophilia related to helminth infection: A pilot study.
PLoS neglected tropical diseasesA case report of right-sided heart failure secondary to JAK2-mutation Loeffler endocarditis.
ESC heart failureEosinophilic Gastroenteritis with Ascites, Elevated Serum IgG4, and Hypereosinophilic Syndrome: A Manifestation of IgG4-related Disease?
Internal medicine (Tokyo, Japan)A unique case of paraneoplastic lymphomatoid Papuloerythroderma of Ofuji with atypical clinical features.
JAAD case reportsManaging Patients with Hypereosinophilic Syndrome: A Statement from the Italian Society of Allergy, Asthma, and Clinical Immunology (SIAAIC).
CellsA case report of Löffler's endocarditis with right ventricular outflow obstruction in idiopathic hypereosinophilic syndrome: timely surgical intervention is crucial.
European heart journal. Case reportsValue and limitations of targeted next-generation sequencing in idiopathic hypereosinophilia: an integrative diagnostic tool in challenging cases.
Clinical and experimental medicineRadioclinical Profile of Eosinophilic Lung: A Case Series.
CureusThe three stages of eosinophilic cardiac damage: A series of case reports.
Journal of cardiology casesTezepelumab for refractory eosinophilic granulomatosis with polyangiitis-related asthma.
Respiratory researchChronic eosinophilic leukemia with a novel JAK1 mutation responds well to the JAK1/2 inhibitor ruxolitinib.
BloodHypereosinophilic syndrome response to mepolizumab in the setting of a compassionate use program.
Journal of leukocyte biologyHypereosinophilia and Left Ventricular Thrombus: A Case Report and Literature Review.
CureusAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
Ainda não temos associações cadastradas para Síndromes hipereosinofílicos.
É de uma associação que acompanha esta doença? Fale com a gente →
Comunidades
Grupos ativos de quem convive com esta doença aqui no Raras
Ainda não existe comunidade no Raras para Síndromes hipereosinofílicos
Pacientes, familiares e cuidadores se organizam em comunidades pra compartilhar experiências, fazer perguntas e se apoiar. Você pode ser o primeiro.
Tire suas dúvidas
Perguntas, dicas e experiências compartilhadas aqui na página
Participe da discussão
Faça login para postar dúvidas, compartilhar experiências e interagir com especialistas.
Fazer loginDoenças relacionadas
Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico
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.
- Long-term outcomes of autologous cell transplantation for patients with non-TAO autoimmune disease-related critical limb ischemia.
- Natural History of Patients With Histologically Proven Acute Eosinophilic Myocarditis.
- Idiopathic Hypereosinophilic Syndrome Presenting as Acute Encephalopathy Secondary to Watershed Ischemic Stroke.
- Idiopathic Hypereosinophilic Syndrome in a Child- Rare Disease with Fulminant Clinical Course.Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood Transfusion· 2026· PMID 41728149mais citado
- Hypereosinophilic syndrome presenting as pleural and pericardial effusion in a Pediatric patient: a rare case report.
- Coronary vasospasm in a patient with RUNX1 mutation in eosinophilic leukemia and hypereosinophilic syndrome: A case report.
- Case Report: Hypereosinophilic syndrome misdiagnosed as atopic dermatitis due to refractory pruritic rash masking peripheral neuropathy.
- Idiopathic Hypereosinophilic Syndrome in a Patient with End-Stage Renal Disease.
- Benralizumab versus placebo for hypereosinophilic syndrome: a randomized, placebo-controlled phase 3 trial.
- Eosinophilic Organ Complications Associated with Dupilumab Therapy - Narrative Review and Current Evidence.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:168956(Orphanet)
- MONDO:0015691(MONDO)
- GARD:2804(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Artigo Wikipedia(Wikipedia)
- Q2551272(Wikidata)
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
