HES
MCID: HYP098
MIFTS: 66

Hypereosinophilic Syndrome (HES)

Categories: Blood diseases, Cancer diseases, Immune diseases, Neuronal diseases, Rare diseases
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Aliases & Classifications for Hypereosinophilic Syndrome

MalaCards integrated aliases for Hypereosinophilic Syndrome:

Name: Hypereosinophilic Syndrome 11 19 58 75 53 14 16 71
Eosinophilia 11 75 28 53 5 43 14 71 31 33
Hes 19 58 47
Disseminated Eosinophilic Collagen Disease 71
Disorders with Increased Eosinophil Counts 33
Hypereosinophilic Syndrome, Idiopathic 19
Idiopathic Hypereosinophilic Syndrome 71
Eosinophilic Leukocytosis 11
Eosinophilic Disorder 71

Characteristics:


Prevelance:

<1/1000000 (United States, Europe) 1-9/100000 (Europe, United States) 58

Age Of Onset:

All ages 58

Classifications:

Orphanet: 58  
Rare haematological diseases


External Ids:

Disease Ontology 11 DOID:999
ICD9CM 34 288.3
MeSH 43 D004802
SNOMED-CT 68 418928000
ICD10 31 D72.1
MESH via Orphanet 44 D017681
ICD10 via Orphanet 32 D47.5
UMLS via Orphanet 72 C1540912
Orphanet 58 ORPHA168956
UMLS 71 C0014457 C0206141 C0263662 more

Summaries for Hypereosinophilic Syndrome

GARD: 19 Hypereosinophilic syndrome (HES) refers to a rare group of conditions that are associated with persistent eosinophilia with evidence of organ involvement. Signs and symptoms vary significantly based on which parts of the body are affected. Although any organ system can be involved in HES, the heart, central nervous system, skin, and respiratory tract are the most commonly affected. The condition was originally thought to be "idiopathic" or of unknown cause. However, recent advances in diagnostic testing have allowed a cause to be identified in approximately a quarter of cases.

MalaCards based summary: Hypereosinophilic Syndrome, also known as eosinophilia, is related to hypereosinophilic syndrome, idiopathic and primary hypereosinophilic syndrome, and has symptoms including nausea and vomiting, constipation and angina pectoris. An important gene associated with Hypereosinophilic Syndrome is FIP1L1 (Factor Interacting With PAPOLA And CPSF1), and among its related pathways/superpathways are Innate Immune System and Signal Transduction. The drugs Prednisone and Benzocaine have been mentioned in the context of this disorder. Affiliated tissues include bone marrow, heart and bone, and related phenotypes are Decreased viability and Increased cell death in breast cancer cell lines (MCF10A, MDA-MB-435)

Orphanet: 58 Hypereosinophilic syndrome (HES) constitutes a rare and heterogeneous group of disorders, defined as persistent and marked blood eosinophilia and/or tissue eosinophilia associated with a wide range of clinical manifestations reflecting eosinophil-induced tissue/organ damage.

Disease Ontology: 11 A leukocyte disease that is characterized by high numbers of eosinophils which over time enter various tissues, eventually damaging organs.

Wikipedia 75 Hypereosinophilic syndrome: Hypereosinophilic syndrome is a disease characterized by a persistently elevated eosinophil count (≥... more...

Eosinophilia: Eosinophilia is a condition in which the eosinophil count in the peripheral blood exceeds 5×108/L... more...

Related Diseases for Hypereosinophilic Syndrome

Diseases in the Hypereosinophilic Syndrome family:

Secondary Hypereosinophilic Syndrome Primary Hypereosinophilic Syndrome

Diseases related to Hypereosinophilic Syndrome via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 1527)
# Related Disease Score Top Affiliating Genes
1 hypereosinophilic syndrome, idiopathic 33.1 PDGFRB PDGFRA KIT IL5 IL3 FIP1L1
2 primary hypereosinophilic syndrome 32.9 PDGFRB PDGFRA FIP1L1 FGFR1
3 loeffler syndrome 32.5 PRG2 IL5 FIP1L1
4 myeloid and lymphoid neoplasms with eosinophilia and abnormalities of pdgfra, pdgfrb, and fgfr1 32.4 PDGFRB PDGFRA FIP1L1 FGFR1
5 paragonimiasis 32.1 RNASE3 IL5 IGHE
6 loeffler endocarditis 32.1 RNASE3 PRG2 PDGFRA IL5 IL3 FIP1L1
7 endomyocardial fibrosis 32.0 RNASE3 PRG2 PDGFRB PDGFRA IL5 FIP1L1
8 aggressive systemic mastocytosis 31.8 PDGFRB PDGFRA KIT JAK2 FIP1L1 ABL1
9 myeloid and lymphoid neoplasms associated with pdgfra rearrangement 31.8 PDGFRB PDGFRA KIT JAK2 FIP1L1 FGFR1
10 pulmonary eosinophilia 31.8 RNASE3 PRG2 IL5 EPX CXCL8 CSF2
11 myeloid and lymphoid neoplasms associated with fgfr1 abnormalities 31.7 IL3 IL2RA FGFR1
12 neutropenia, severe congenital, 3, autosomal recessive 31.7 IL3 CSF2
13 restrictive cardiomyopathy 31.3 RNASE3 PDGFRA FIP1L1 EPX
14 chronic eosinophilic leukemia 31.3 PDGFRB PDGFRA KIT JAK2 IL5 IL3
15 strongyloidiasis 31.1 IL5 IGHE CCL11
16 exanthem 31.1 KIT IFNA1 CXCL8 CSF2
17 neutrophilia, hereditary 31.0 JAK2 IL3 CSF2
18 aspergillosis 31.0 IL5 EPX CXCL8 CSF2
19 peripheral t-cell lymphoma 31.0 PDGFRB PDGFRA IL2RA ABL1
20 churg-strauss syndrome 31.0 RNASE3 PRG2 IL5 IL3 IL2RA CXCL8
21 peripheral nervous system disease 30.9 KIT JAK2 IFNA1 CXCL8 CSF2
22 budd-chiari syndrome 30.9 PDGFRA JAK2 FIP1L1
23 mastocytosis, cutaneous 30.9 PDGFRA KIT IL3 FIP1L1
24 systemic mastocytosis 30.8 PDGFRB PDGFRA KIT JAK2 IL2RA FIP1L1
25 mastocytosis 30.8 PDGFRB PDGFRA KIT JAK2 IL3 IL2RA
26 leukemia, acute lymphoblastic 30.8 KIT JAK2 IL3 CSF2 ABL1
27 toxocariasis 30.8 RNASE3 IL5 IGHE FIP1L1
28 diarrhea 30.8 IL5 IL3 IFNA1 CXCL8
29 leukemia 30.7 PDGFRB KIT JAK2 IL3 FGFR1 CSF2
30 eosinophilic gastroenteritis 30.7 RNASE3 IL5 IL3 IGHE FIP1L1 EPX
31 angiosarcoma 30.7 PDGFRB PDGFRA KIT ABL1
32 granulocytopenia 30.7 IL3 CSF2
33 eosinophilic pneumonia 30.7 RNASE3 IL5 IL3 FIP1L1 CXCL8 CSF2
34 myeloproliferative neoplasm 30.7 ZBTB20 PDGFRB PDGFRA KIT JAK2 IL3
35 dermatitis 30.7 RNASE3 IL5 IGHE IGES CXCL8 CCL11
36 proteasome-associated autoinflammatory syndrome 1 30.7 RNASE3 IL5 IGHE IFNA1 CXCL8 CCL11
37 urticaria 30.7 RNASE3 PRG2 KIT IL5 IL3 IGHE
38 pemphigoid 30.7 RNASE3 IL5 CXCL8 CCL11
39 myelofibrosis 30.6 PDGFRB KIT JAK2 IL3 IL2RA IFNA1
40 kimura disease 30.6 RNASE3 IL5 IGHE IGES
41 primary cutaneous t-cell non-hodgkin lymphoma 30.6 IL5 IL2RA IFNA1
42 leukemia, chronic myeloid 30.6 PDGFRB PDGFRA KIT JAK2 IL3 IFNA1
43 eosinophilic gastritis 30.6 IL5 FIP1L1 EPX CCL11
44 dermatographia 30.6 PRG2 IGHE
45 trichinosis 30.6 IL5 IL3 CCL11
46 cellulitis 30.6 RNASE3 IL5 IGES CXCL8 CSF2
47 acute leukemia 30.6 KIT JAK2 IL3 CSF2
48 childhood-onset asthma 30.6 RNASE3 IL5 IGHE IGES EPX CCL11
49 bronchiolitis 30.6 RNASE3 IL5 IFNA1 CXCL8 CSF2 CCL11
50 bullous pemphigoid 30.6 RNASE3 IL5 IGHE CXCL8 CCL11

Comorbidity relations with Hypereosinophilic Syndrome via Phenotypic Disease Network (PDN):


Acute Cystitis Bronchitis
Deficiency Anemia Heart Disease
Hypertension, Essential

Graphical network of the top 20 diseases related to Hypereosinophilic Syndrome:



Diseases related to Hypereosinophilic Syndrome

Symptoms & Phenotypes for Hypereosinophilic Syndrome

UMLS symptoms related to Hypereosinophilic Syndrome:


nausea and vomiting; constipation; angina pectoris; abdominal pain; myalgia; chest pain; pruritus; diarrhea; edema; snoring; dyspepsia; icterus; coughing; heartburn; gastrointestinal gas

GenomeRNAi Phenotypes related to Hypereosinophilic Syndrome according to GeneCards Suite gene sharing:

25 (show all 16)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased viability GR00173-A 9.98 PDGFRA
2 Decreased viability GR00221-A-1 9.98 ABL1 FGFR1 KIT PDGFRA PDGFRB
3 Decreased viability GR00221-A-2 9.98 ABL1 FGFR1
4 Decreased viability GR00221-A-3 9.98 ABL1 PDGFRA PDGFRB
5 Decreased viability GR00221-A-4 9.98 PDGFRA PDGFRB
6 Decreased viability GR00249-S 9.98 PDGFRA
7 Decreased viability GR00301-A 9.98 KIT
8 Decreased viability GR00342-S-1 9.98 ABL1 PDGFRB
9 Decreased viability GR00342-S-2 9.98 ABL1
10 Decreased viability GR00342-S-3 9.98 ABL1
11 Decreased viability GR00386-A-1 9.98 FGFR1
12 Decreased viability GR00402-S-2 9.98 PDGFRA
13 Increased cell death in breast cancer cell lines (MCF10A, MDA-MB-435) GR00104-A-0 9.46 CSF2 CXCL8 IL2RA PDGFRB
14 Decreased substrate adherent cell growth GR00193-A-1 9.1 KIT
15 Decreased substrate adherent cell growth GR00193-A-2 9.1 ABL1 KIT
16 Decreased substrate adherent cell growth GR00193-A-4 9.1 ABL1 FGFR1 KIT

MGI Mouse Phenotypes related to Hypereosinophilic Syndrome:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 immune system MP:0005387 10.06 ABL1 CCL11 CSF2 EPX FGFR1 IFNA1
2 respiratory system MP:0005388 9.7 ABL1 CCL11 CSF2 IFNA1 IL2RA IL3
3 hematopoietic system MP:0005397 9.53 ABL1 CCL11 CSF2 EPX FGFR1 IFNA1

Drugs & Therapeutics for Hypereosinophilic Syndrome

Drugs for Hypereosinophilic Syndrome (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 186)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Prednisone Approved, Vet_approved Phase 4 53-03-2 5865
2
Benzocaine Approved, Investigational Phase 4 1994-09-7, 94-09-7 2337
3
Tannic acid Approved Phase 4 1401-55-4 16129878 16129778
4
Prednisolone phosphate Approved, Vet_approved Phase 4 302-25-0
5
Prednisolone acetate Approved, Vet_approved Phase 4 52-21-1
6
Prednisolone Approved, Vet_approved Phase 4 50-24-8 4894 5755
7
Methylprednisolone hemisuccinate Approved Phase 4 2921-57-5 1875
8
Methylprednisolone Approved, Vet_approved Phase 4 83-43-2 4159 6741
9
Azithromycin Approved Phase 4 83905-01-5 447043
10
Salbutamol Approved, Vet_approved Phase 4 18559-94-9 2083
11
Budesonide Approved Phase 4 51333-22-3 5281004 40000 63006
12
Prednisolone hemisuccinate Experimental Phase 4 2920-86-7 4897
13 Protective Agents Phase 4
14 Anti-Allergic Agents Phase 4
15 Neuroprotective Agents Phase 4
16 Antiemetics Phase 4
17 Gastrointestinal Agents Phase 4
18 Antineoplastic Agents, Hormonal Phase 4
19
Methylprednisolone Acetate Phase 4 584547
20 Hormones Phase 4
21 Hormone Antagonists Phase 4
22 glucocorticoids Phase 4
23 Anti-Inflammatory Agents Phase 4
24 Neurotransmitter Agents Phase 4
25 Bronchodilator Agents Phase 4
26 Adrenergic beta-Agonists Phase 4
27 Adrenergic Agonists Phase 4
28 Adrenergic Agents Phase 4
29 Tocolytic Agents Phase 4
30 Budesonide, Formoterol Fumarate Drug Combination Phase 4
31 Formoterol Fumarate Phase 4
32
Lenograstim Approved, Investigational Phase 3 135968-09-1
33
Asparaginase Escherichia coli Approved, Investigational Phase 3 9015-68-3
34
Tioguanine Approved Phase 3 154-42-7 2723601
35
Daunorubicin Approved Phase 3 20830-81-3 30323
36
Busulfan Approved, Investigational Phase 3 55-98-1 2478
37
Aldesleukin Approved Phase 3 110942-02-4
38
Benralizumab Approved, Investigational Phase 3 1044511-01-4
39
Ketotifen Approved Phase 3 34580-14-8, 34580-13-7 3827
40
Histamine Approved, Investigational Phase 3 51-45-6 774
41
Reslizumab Approved, Investigational Phase 3 241473-69-8
42
Aspartic acid Approved, Nutraceutical Phase 3 56-84-8 5960
43 Alkylating Agents Phase 3
44 Antineoplastic Agents, Alkylating Phase 3
45 N-Methylaspartate Phase 3
46 Anti-Retroviral Agents Phase 3
47 Interleukin-2 Phase 3
48 Anti-HIV Agents Phase 3
49 Analgesics, Non-Narcotic Phase 3
50 Calcium, Dietary Phase 3

Interventional clinical trials:

(show top 50) (show all 207)
# Name Status NCT ID Phase Drugs
1 How to "Choosebetweenamab" for Severe Asthma, Comparing Treatment With Mepolizumab and Omalizumab for Patients With Severe Allergic and Eosinophilic Asthma. Unknown status NCT04585997 Phase 4 Mepolizumab;Omalizumab
2 A Pragmatic Study to Investigate the Efficacy and Safety of Mepolizumab in Severe Uncontrolled Asthma in Brazil Unknown status NCT04228588 Phase 4 Mepolizumab 100 MG [Nucala]
3 Impact of Benralizumab Treatment on Circulating Dendritic Cells in Patients With Eosinophilic Asthma Unknown status NCT03652376 Phase 4 Benralizumab
4 Assessment of Glucocorticoid Responsiveness and Mechanisms of Resistance in Hypereosinophilic Syndromes Completed NCT01524536 Phase 4 prednisone
5 Phase 4, Randomized Study of Three Months-prednisolone Therapy in the Treatment of Chronic Eosinophilic Pneumonia Completed NCT00632554 Phase 4 prednisolone 0.5 mg/kg/day for three months;prednisolone 0.5 mg/kg/day for six months
6 A Multi-centre, Open Label, Single Arm, 32-week Treatment Study in Subjects With Severe Eosinophilic Asthma Not Optimally Controlled With Current Omalizumab Treatment Who Are Switched From Omalizumab to Mepolizumab 100mg Subcutaneous (Study Number 204471- the OSMO Study) Completed NCT02654145 Phase 4 Mepolizumab 100mg SC;Albuterol/salbutamol MDIs;Omalizumab
7 Efficacy of Reslizumab Dose Escalation in Patients With Severe Asthma and Persistent Sputum Eosinophilia Despite Standard Dose Therapy Recruiting NCT04710134 Phase 4
8 BURAN: Effects of Benralizumab on Airway Dynamics in Severe Eosinophilic Asthma Using Functional Respiratory Imaging Parameters Recruiting NCT05552508 Phase 4
9 A Phase 4, Open-label, Single Arm, 24-week, Phase 4 Study to Evaluate the Safety and Efficacy of Mepolizumab 100 mg SC Administered Every 4 Weeks in Indian Participants Aged >=18 Years With Severe Eosinophilic Asthma Requiring Oral Corticosteroid Treatment to Maintain Asthma Control (PRISM) Recruiting NCT04276233 Phase 4 Prednisone/ Prednisolone;Salbutamol
10 Effects of Azithromycin Treatment on Anti-viral Immunity in Patients With Asthma and COPD - A Randomized Double-blind, Placebo-controlled Trial Recruiting NCT04319705 Phase 4 Azithromycin;Placebo oral tablet
11 Predictive Signature of Benralizumab Response Recruiting NCT04565483 Phase 4 Benralizumab Prefilled Syringe
12 SHAMAL: A Multicentre, Randomised, Open-Label, Parallel-Group, Active-Controlled, Phase IV Study to Assess the Reduction of Daily Maintenance ICS/LABA Treatment Towards Anti-Inflammatory Reliever Treatment in Patients With Severe Eosinophilic Asthma Treated With Benralizumab Active, not recruiting NCT04159519 Phase 4 Symbicort®;Fasenra®;Ventolin®
13 Route of Administration of Anti-IL5 Monoclonal Antibody in Prednisone-dependent Eosinophilic Asthma Completed NCT02559791 Phase 2, Phase 3 Placebo
14 A Three-part,Multicenter Study,With a Randomized,Double-blind,Placebo Controlled,Withdrawal Design in Part II to Assess Efficacy,Safety,and Tolerability of ACZ885(Anti-interleukin-1beta Monoclonal Antibody)in Patients With Muckle-Wells Syndrome Completed NCT00465985 Phase 3 ACZ885;Placebo
15 An Open-label Study to Evaluate the Pharmacokinetics and Pharmacodynamics and Long-term Safety of Benralizumab Administered Subcutaneously in Children With Severe Eosinophilic Asthma Completed NCT04305405 Phase 3 Benralizumab
16 A Multi-center, Randomized, Double-blind, Placebo Controlled, Parallel Group Study to Compare Cessation Versus Continuation of Long-term Mepolizumab Treatment in Patients With Severe Eosinophilic Asthma (201810) Completed NCT02555371 Phase 3 Placebo
17 An Open-label, Long-term Safety and Efficacy Study of ACZ885 (Anti-interleukin-1β Monoclonal Antibody) Administered for at Least 6 Months in Patients With the Following Cryopyrin-associated Periodic Syndromes: Familial Cold Autoinflammatory Syndrome, Muckle-Wells Syndrome, or Neonatal Onset Multisystem Inflammatory Disease Completed NCT00685373 Phase 3 Canakinumab (ACZ885)
18 A Randomised, Double-blind, Placebo-controlled, Parallel-group, Multi-centre 24-week Study to Evaluate the Efficacy and Safety of Mepolizumab Adjunctive Therapy in Subjects With Severe Eosinophilic Asthma on Markers of Asthma Control Completed NCT02281318 Phase 3 Placebo;SOC
19 An Open-label, Efficacy and Safety Study of Canakinumab (Anti-interleukin-1β Monoclonal Antibody) Administered for 6 Months (24 Weeks) in Japanese Patients With the Following Cryopyrin-associated Periodic Syndromes: Familial Cold Autoinflammatory Syndrome, Muckle-Wells Syndrome, or Neonatal Onset Multisystem Inflammatory Disease, Followed by an Extension Phase to Provide Canakinumab to Study Patients Until it is Approved and Marketed in Japan Completed NCT00991146 Phase 3 canakinumab
20 A 16-Week, Randomized, Double-Blind, Placebo-Controlled, Parallel-Group Study to Evaluate the Efficacy and Safety of Reslizumab (0.3 or 3.0 mg/kg) as Treatment for Patients (12-75 Years of Age) With Eosinophilic Asthma Completed NCT01270464 Phase 3 Reslizumab;Placebo
21 An Open-label Extension Study to Assess Efficacy, Safety and Tolerability of Canakinumab and the Efficacy and Safety of Childhood Vaccinations in Patients With Cryopyrin Associated Periodic Syndromes (CAPS) Completed NCT01576367 Phase 3
22 A 12-Month, Double-Blind, Placebo-Controlled, Parallel-Group Study to Evaluate the Efficacy and Safety of Reslizumab (3.0 mg/kg) in the Reduction of Clinical Asthma Exacerbations in Patients (12-75 Years of Age) With Eosinophilic Asthma Completed NCT01287039 Phase 3 Reslizumab;Placebo
23 A Randomized, Double-Blind, Placebo-controlled Clinical Trial to Evaluate the Efficacy of Benralizumab (Anti-IL5RA) in Subjects With Eosinophilic Gastritis Completed NCT03473977 Phase 2, Phase 3
24 A 12-Month, Double-Blind, Placebo-Controlled, Parallel-Group Study to Evaluate the Efficacy and Safety of Reslizumab (3.0 mg/kg) in the Reduction of Clinical Asthma Exacerbations in Patients (12-75 Years of Age) With Eosinophilic Asthma Completed NCT01285323 Phase 3 Reslizumab;Placebo
25 A 16-Week, Randomized, Double-Blind, Placebo-Controlled Study to Evaluate the Efficacy and Safety of Reslizumab (3.0 mg/kg) Treatment in Patients With Moderate to Severe Asthma Completed NCT01508936 Phase 3 Reslizumab;Placebo
26 A One-year Open-label, Multicenter Trial to Assess Efficacy, Safety and Tolerability of Canakinumab (ACZ885) and the Efficacy and Safety of Childhood Vaccinations in Patients Aged 4 Years or Younger With Cryopyrin Associated Periodic Syndromes (CAPS) Completed NCT01302860 Phase 3 ACZ885
27 A Multi-centre, Open-label Extension, Safety Study to Describe the Long-term Clinical Experience of Mepolizumab in Participants With Hypereosinophilic Syndrome (HES) From Study 200622 Completed NCT03306043 Phase 3 Mepolizumab
28 Study 200622: A Randomized, Double-blind, Placebo-controlled Study to Investigate the Efficacy and Safety of Mepolizumab in the Treatment of Adolescent and Adult Subjects With Severe Hypereosinophilic Syndrome Completed NCT02836496 Phase 3 Mepolizumab 300 mg;Placebo matching mepolizumab;Active OCS capsules (5 mg prednisolone or prednisone);Placebo matching OCS capsules
29 A Multicenter, Double-blind, Placebo-controlled, Study to to Evaluate the Corticosteroid- Sparing Effects of Mepolizumab in Subjects With Hypereosinophilic Syndromes (HES) and Evaluate Efficacy and Safety of Mepolizumab in Controlling the Clinical Signs and Symptoms of Subjects With HES Completed NCT00086658 Phase 3 mepolizumab
30 INSPIRE: An Internet-based RCT for Long-term Survivors of Hematopoietic Stem Cell Transplantation Completed NCT00799461 Phase 3
31 The Treatment of Down Syndrome Children With Acute Myeloid Leukemia (AML) and Myelodysplastic Syndromes (MDS) Under the Age of 4 Years Completed NCT00369317 Phase 3 asparaginase;daunorubicin hydrochloride;cytarabine;thioguanine;etoposide
32 Phase III Randomized Study of Induction Chemotherapy With or Without MDR-Modulation With PSC-833 (NSC # 648265, IND # 41121) Followed by Cytogenetic Risk-Adapted Intensification Therapy Followed by Immunotherapy With rIL-2 (NSC # 373364, IND # 1969) vs. Observation in Previously Untreated Patients With AML < 60 Years Completed NCT00006363 Phase 3 cytarabine;daunorubicin hydrochloride;etoposide;valspodar;busulfan
33 A Randomized Double Blinded Trial of Topical Caphosol to Prevent Oral Mucositis in Children Undergoing Hematopoietic Stem Cell Transplantation Completed NCT01305200 Phase 3 supersaturated calcium phosphate rinse
34 A Randomised Controlled Trial of Mepolizumab Initiated Following Admission to Hospital for a Severe Exacerbation of Eosinophilic COPD Recruiting NCT04075331 Phase 2, Phase 3 Mepolizumab;Placebo
35 Double-blind, Placebo-controlled, Cross-over Trial of Ketotifen in Children and Adolescents With Functional Dyspepsia in Association With Duodenal Eosinophilia Recruiting NCT02484248 Phase 3 Ketotifen;Placebo
36 A Phase 3, Multicenter, Randomized, Double-blind, Placebo-controlled Induction and Maintenance Study to Evaluate the Efficacy and Safety of CC-93538 in Adult and Adolescent Japanese Subjects With Eosinophilic Gastroenteritis Recruiting NCT05214768 Phase 3 CC-93538;Placebo
37 A Multi-center, Randomized, Double-blind, Parallel-group, Placebo-controlled 3-Part Phase 3 Study to Demonstrate the Efficacy and Safety of Benralizumab in Patients With Eosinophilic Gastritis and/or Gastroenteritis (The HUDSON GI Study) Recruiting NCT05251909 Phase 3
38 A Phase 3 Multicenter, Randomized, Double-Blind, Placebo-Controlled Study to Evaluate the Efficacy, Safety, Tolerability, and Pharmacodynamic Effect of Subcutaneous AK002 in Subjects With Moderate to Severe Eosinophilic Gastritis and/or Eosinophilic Duodenitis Recruiting NCT05152563 Phase 3 AK002
39 Benralizumab in Patients With Inadequate Response to Anti-IL5 Monoclonal Antibody Therapies Recruiting NCT03470311 Phase 3
40 A Multicentre, Randomised, Double-blind, Parallel-group, Placebo-controlled, 24-week Phase 3 Study With an Open-label Extension to Evaluate the Efficacy and Safety of Benralizumab in Patients With Hypereosinophilic Syndrome (HES) Recruiting NCT04191304 Phase 3
41 A Randomized, Double-blind, Placebo-controlled Study to Investigate the Efficacy and Safety of Depemokimab in Adults With Hypereosinophilic Syndrome (HES) Recruiting NCT05334368 Phase 3 Depemokimab
42 A Phase 3, 52-week, Open-label, Single Arm Study to Investigate the Efficacy and Safety of Mepolizumab SC in Participants Aged 6 to 17 Years With Hypereosinophilic Syndrome Recruiting NCT04965636 Phase 3 Mepolizumab
43 A Phase 2a Randomized, Double-Blind, Placebo-Controlled Study to Evaluate the Safety and Efficacy of Subcutaneous Benralizumab (MEDI-563) in Reducing Eosinophilia in Subjects With Hypereosinophilic Syndrome (HES) Active, not recruiting NCT02130882 Phase 2, Phase 3 benralizumab
44 A Phase 3, Multicenter, Randomized, Double-Blind, Placebo-Controlled Study to Evaluate Efficacy and Safety of AK002 in Patients With Moderately to Severely Active Eosinophilic Gastritis and/or Eosinophilic Duodenitis (Formerly Referred to as Eosinophilic Gastroenteritis) Active, not recruiting NCT04322604 Phase 3 lirentelimab (AK002)
45 A Phase 3, Multicenter, Open-Label, Extension Study to Evaluate the Efficacy and Safety of AK002 in Patients That Were Previously Enrolled in AK002-016 or AK002-012 Studies and Have Eosinophilic Gastritis and/or Eosinophilic Duodenitis (Formerly Referred to as Eosinophilic Gastroenteritis) Enrolling by invitation NCT04620811 Phase 3 lirentelimab
46 Treatment of DRESS (Drug Reaction With Eosinophilia and Systemic Symptoms) With Tegeline® Terminated NCT00505648 Phase 3 Tegeline®
47 An Open-Label Extension Study of Reslizumab 110-mg Fixed, Subcutaneous Dosing in Patients 12 Years of Age and Older With Severe Eosinophilic Asthma Terminated NCT03052725 Phase 3 reslizumab
48 A Randomized, Double-blind, Placebo-controlled, Mono-center Study to Evaluate the Effects of Mepolizumab on Airway Physiology in Patients With Eosinophilic Asthma: the MEMORY Study Terminated NCT02594332 Phase 3 Mepolizumab;Placebo
49 A 52 Week, Randomised, Double Blind, Multinational, Multicentre, 4-arm Parallel Group Trial to Assess the Efficacy and Safety of 3 Doses of CHF 6532 Compared to Placebo on Top of Standard of Care in Patients With Uncontrolled Severe Eosinophilic Asthma Terminated NCT04049175 Phase 3 Treatment A;Treatment B;Treatment C;Treatment D
50 An Open-Label Extension Study to Evaluate the Long-Term Safety and Efficacy of Reslizumab (3.0 mg/kg) as Treatment for Patients With Eosinophilic Asthma Who Completed a Prior Teva-Sponsored Study in Eosinophilic Asthma Terminated NCT01290887 Phase 3 Reslizumab

Search NIH Clinical Center for Hypereosinophilic Syndrome

Inferred drug relations via UMLS 71 / NDF-RT 50 :


hydroxyurea
imatinib
Imatinib mesylate
Interferon Alfa-2a
Interferon Alfa-2b
INTERFERON ALFA-3N,HUMAN LEUKOCYTE DERIVED
interferon alfacon-1
Interferon gamma-1b
Interferons
peginterferon alfa-2a
peginterferon alfa-2b
Recombinant interferon beta-1a
Recombinant interferon beta-1b

Cochrane evidence based reviews: eosinophilia

Genetic Tests for Hypereosinophilic Syndrome

Genetic tests related to Hypereosinophilic Syndrome:

# Genetic test Affiliating Genes
1 Eosinophilia 28

Anatomical Context for Hypereosinophilic Syndrome

Organs/tissues related to Hypereosinophilic Syndrome:

MalaCards : Bone Marrow, Heart, Bone, Myeloid, Skin, Lung, Liver

Publications for Hypereosinophilic Syndrome

Articles related to Hypereosinophilic Syndrome:

(show top 50) (show all 23052)
# Title Authors PMID Year
1
Delayed-type asthmatic response to bronchial challenge with allergen, I: clinical features. 53 62
20486329 2010
2
Early phase resolution of mucosal eosinophilic inflammation in allergic rhinitis. 53 62
20459697 2010
3
Thrombotic storm in Kimura disease. 53 62
19468829 2010
4
IL-13 Gene Polymorphisms are Associated With Rhinosinusitis and Eosinophilic Inflammation in Aspirin Intolerant Asthma. 53 62
20358028 2010
5
Airway wall expression of OX40/OX40L and interleukin-4 in asthma. 53 62
20139223 2010
6
Allergen-induced, eotaxin-rich, proangiogenic bone marrow progenitors: a blood-borne cellular envoy for lung eosinophilia. 53 62
20227754 2010
7
Critical role of the platelet-derived growth factor receptor (PDGFR) beta transmembrane domain in the TEL-PDGFRbeta cytosolic oncoprotein. 53 62
20164181 2010
8
Hypereosinophilic syndrome and clonal eosinophilia: point-of-care diagnostic algorithm and treatment update. 53 62
20053713 2010
9
Vernal keratoconjunctivitis--a rare but serious comorbidity of allergic rhinitis and eustachian tube dysfunction. 53 62
19900723 2010
10
Hyper-IgE syndrome with STAT3 mutation: a case report in Mainland China. 53 62
20490271 2010
11
Intranasal immunization of mice with a bovine respiratory syncytial virus vaccine induces superior immunity and protection compared to those by subcutaneous delivery or combinations of intranasal and subcutaneous prime-boost strategies. 53 62
19864487 2010
12
A diagnostically difficult case of chronic myeloid neoplasm with eosinophilia and abnormalities of PDGFRA effectively treated with imatinib in accelerated phase: case report. 53 62
20010473 2009
13
Effects of omalizumab on markers of inflammation in patients with allergic asthma. 53 62
19839977 2009
14
Concomitant occurrence of kimura disease and mycosis fungoides in a Lebanese woman: significance and response to rituximab. 53 62
19786854 2009
15
Myeloproliferative neoplasms: contemporary diagnosis using histology and genetics. 53 62
19806146 2009
16
Kimura's disease presenting with a giant suspensory tumor and associated with membranoproliferative glomerulonephritis. 53 62
19620037 2009
17
A low dose of Mycoplasma pneumoniae infection enhances an established allergic inflammation in mice: the role of the prostaglandin E2 pathway. 53 62
19552640 2009
18
Triptolide abrogates oncogene FIP1L1-PDGFRalpha addiction and induces apoptosis in hypereosinophilic syndrome. 53 62
19671059 2009
19
Leukotriene E4-induced pulmonary inflammation is mediated by the P2Y12 receptor. 53 62
19822647 2009
20
Recurrent giant chalazia in hyperimmunoglobulin E (Job's) syndrome. 53 62
18528638 2009
21
Is serum total IgE levels a good predictor of allergies in children? 53 62
19813686 2009
22
T-helper type 2-driven inflammation defines major subphenotypes of asthma. 53 62
19483109 2009
23
Changed concepts and definitions of myeloproliferative neoplasms (MPN), myelodysplastic syndromes (MDS) and myelodysplastic/myeloproliferative neoplasms (MDS/MPN) in the updated 2008 WHO classification. 53 62
20309429 2009
24
Innate cells and T helper 2 cell immunity in airway inflammation. 53 62
19766085 2009
25
Recent breakthroughs in the understanding and management of chronic eosinophilic leukemia. 53 62
19761433 2009
26
Formulation of bovine respiratory syncytial virus fusion protein with CpG oligodeoxynucleotide, cationic host defence peptide and polyphosphazene enhances humoral and cellular responses and induces a protective type 1 immune response in mice. 53 62
19386785 2009
27
Similar colds in subjects with allergic asthma and nonatopic subjects after inoculation with rhinovirus-16. 53 62
19596142 2009
28
Blockade of CCR4 in a humanized model of asthma reveals a critical role for DC-derived CCL17 and CCL22 in attracting Th2 cells and inducing airway inflammation. 53 62
19630858 2009
29
Production of interleukin-10 in asthmatic children after Beta-1-3-glucan. 53 62
19912977 2009
30
Clinical significance of eosinophilic cationic protein levels in nasal secretions of patients with nasal polyposis. 53 62
19034473 2009
31
Imatinib has limited therapeutic activity for hypereosinophilic syndrome patients with unknown or negative PDGFRalpha mutation status. 53 62
19013640 2009
32
A novel FIP1L1-PDGFRA mutant destabilizing the inactive conformation of the kinase domain in chronic eosinophilic leukemia/hypereosinophilic syndrome. 53 62
19210352 2009
33
Pathogenesis of Churg-Strauss syndrome: recent insights. 53 62
19811306 2009
34
Eosinophilia-associated muscle disorders: an immunohistological study with tissue localisation of major basic protein in distinct clinicopathological forms. 53 62
19139036 2009
35
A scleroderma-like cutaneous syndrome associated with a marked Th2-type immune response occurring after a prosthetic joint implant. 53 62
19300283 2009
36
IL-25: a key requirement for the regulation of type-2 immunity. 53 62
19449446 2009
37
VCAM-1 and eosinophilia in diffuse sino-nasal polyps. 53 62
18642014 2009
38
Molecular drug targets in myeloproliferative neoplasms: mutant ABL1, JAK2, MPL, KIT, PDGFRA, PDGFRB and FGFR1. 53 62
19175693 2009
39
Reciprocal regulation of iNOS and PARP-1 during allergen-induced eosinophilia. 53 62
18829681 2009
40
Evidence that 13-14 di-hydro, 15-keto prostaglandin D(2)-induced airway eosinophilia in guinea-pigs is independent of interleukin-5. 53 62
19184361 2009
41
Kimura's disease in a young Balkan male. 53 62
19195268 2009
42
Th2 cells as targets for therapeutic intervention in allergic bronchial asthma. 53 62
19099351 2009
43
Thrombosis of temporal artery and renal vein in Kimura-disease-related nephrotic syndrome. 53 62
18066701 2009
44
Sputum IL-5 concentration is associated with a sputum eosinophilia and attenuated by corticosteroid therapy in COPD. 53 62
19478474 2009
45
Five years since the discovery of FIP1L1-PDGFRA: what we have learned about the fusion and other molecularly defined eosinophilias. 53 62
18843283 2008
46
Immunosuppression of TH2 responses in Trichinella spiralis infection by Helicobacter pylori neutrophil-activating protein. 53 62
18804852 2008
47
TARC and IL-5 expression correlates with tissue eosinophilia in peripheral T-cell lymphomas. 53 62
18395252 2008
48
Liver X receptor agonists increase airway reactivity in a model of asthma via increasing airway smooth muscle growth. 53 62
18768884 2008
49
CD4+CD25+ regulatory T cells reverse established allergic airway inflammation and prevent airway remodeling. 53 62
18672278 2008
50
Treatment with anti-CC chemokine receptor 3 monoclonal antibody or dexamethasone inhibits the migration and differentiation of bone marrow CD34 progenitor cells in an allergic mouse model. 53 62
18699933 2008

Variations for Hypereosinophilic Syndrome

ClinVar genetic disease variations for Hypereosinophilic Syndrome:

5
# Gene Name Type Significance ClinVarId dbSNP ID Position
1 JAK2, ZBTB20 t(3;9)(q13.31;p24.1) TRANS Likely Pathogenic
869413 GRCh37: 3:114069121-114069122
GRCh38:

Expression for Hypereosinophilic Syndrome

Search GEO for disease gene expression data for Hypereosinophilic Syndrome.

Pathways for Hypereosinophilic Syndrome

Pathways related to Hypereosinophilic Syndrome according to GeneCards Suite gene sharing:

(show top 50) (show all 53)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.95 ABL1 CCL11 CSF2 CXCL8 EPX IFNA1
2 13.73 PDGFRB PDGFRA KIT JAK2 IL5 IL3
3
Show member pathways
13.61 PRG2 PDGFRB PDGFRA KIT JAK2 IL5
4
Show member pathways
13.52 CCL11 CSF2 CXCL8 FGFR1 IFNA1 IL2RA
5
Show member pathways
13.47 PDGFRB PDGFRA KIT JAK2 IL5 IL3
6
Show member pathways
13.31 JAK2 IL5 IL3 IL2RA IFNA1 CXCL8
7
Show member pathways
13.3 PDGFRB PDGFRA KIT JAK2 IL5 IL3
8
Show member pathways
13.14 CCL11 CSF2 CXCL8 FGFR1 IL2RA IL3
9
Show member pathways
13.06 PRG2 PDGFRB PDGFRA KIT IL3 IL2RA
10
Show member pathways
13.03 PDGFRB PDGFRA KIT JAK2 IL3 IL2RA
11
Show member pathways
12.96 PDGFRB PDGFRA KIT JAK2 IL5 IL3
12
Show member pathways
12.92 FGFR1 IFNA1 IL2RA IL3 JAK2 KIT
13 12.82 PDGFRB PDGFRA KIT JAK2 FGFR1
14 12.68 PDGFRB PDGFRA KIT FGFR1 CSF2
15 12.56 KIT JAK2 IL3 IL2RA IFNA1
16
Show member pathways
12.44 PDGFRB PDGFRA KIT FGFR1
17
Show member pathways
12.44 FGFR1 IL2RA IL5 PDGFRA PDGFRB
18
Show member pathways
12.37 PDGFRB PDGFRA KIT IL3 FGFR1
19
Show member pathways
12.28 JAK2 IL5 IL3 IL2RA CSF2
20 12.24 ABL1 FGFR1 KIT PDGFRA PDGFRB
21
Show member pathways
12.19 PDGFRB PDGFRA IL2RA FGFR1
22 12.02 PDGFRB PDGFRA KIT JAK2 FGFR1 ABL1
23 11.99 KIT IL5 IL3 IL2RA CSF2
24 11.92 JAK2 CXCL8 CCL11
25 11.88 PDGFRA IL5 IL2RA CXCL8
26
Show member pathways
11.87 IL2RA IL3 JAK2
27
Show member pathways
11.87 PDGFRB PDGFRA KIT JAK2 IL5 IL3
28
Show member pathways
11.86 PDGFRB PDGFRA KIT FGFR1
29
Show member pathways
11.85 JAK2 CXCL8 CSF2
30 11.83 PDGFRB PDGFRA FGFR1
31 11.77 KIT IL5 IL2RA
32
Show member pathways
11.76 FGFR1 KIT PDGFRB
33 11.75 IL5 CXCL8 CSF2
34 11.74 PDGFRB FGFR1 CXCL8
35
Show member pathways
11.73 JAK2 IL5 IL3 CSF2
36 11.7 PDGFRB PDGFRA IL3 FGFR1
37 11.69 IL5 CXCL8 CSF2
38
Show member pathways
11.67 PDGFRB PDGFRA KIT ABL1
39 11.66 IL5 CXCL8 CSF2 CCL11
40 11.63 JAK2 IL5 CCL11
41
Show member pathways
11.63 IL5 IL3 IL2RA CXCL8 CSF2
42 11.58 PDGFRB JAK2 IL2RA
43 11.51 JAK2 IL5 IL2RA CCL11
44 11.5 PDGFRB JAK2 ABL1
45 11.49 PDGFRB PDGFRA FGFR1
46 11.46 FGFR1 PDGFRA PDGFRB
47
Show member pathways
11.35 PDGFRB PDGFRA KIT
48 11.28 IL5 IL3 CSF2
49 11.25 IL5 CXCL8 CSF2
50 11.06 PDGFRB PDGFRA KIT ABL1

GO Terms for Hypereosinophilic Syndrome

Cellular components related to Hypereosinophilic Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 10.16 RNASE3 KIT IL5 IL3 IGHE IFNA1
2 extracellular region GO:0005576 10 CCL11 CSF2 CXCL8 EPX FGFR1 IFNA1
3 granulocyte macrophage colony-stimulating factor receptor complex GO:0030526 8.92 JAK2 CSF2

Biological processes related to Hypereosinophilic Syndrome according to GeneCards Suite gene sharing:

(show all 31)
# Name GO ID Score Top Affiliating Genes
1 protein phosphorylation GO:0006468 10.25 ABL1 CCL11 CSF2 FGFR1 JAK2 KIT
2 cytokine-mediated signaling pathway GO:0019221 10.24 KIT JAK2 IL5 IFNA1
3 positive regulation of cell migration GO:0030335 10.23 PDGFRB PDGFRA KIT JAK2 CSF2 CCL11
4 transmembrane receptor protein tyrosine kinase signaling pathway GO:0007169 10.22 PDGFRB PDGFRA KIT FGFR1
5 positive regulation of cell population proliferation GO:0008284 10.22 PDGFRB PDGFRA KIT JAK2 IL5 IL3
6 positive regulation of peptidyl-tyrosine phosphorylation GO:0050731 10.15 ABL1 IL3 IL5 JAK2
7 protein autophosphorylation GO:0046777 10.15 ABL1 FGFR1 JAK2 KIT PDGFRA PDGFRB
8 positive regulation of kinase activity GO:0033674 10.14 PDGFRB PDGFRA KIT FGFR1
9 positive regulation of fibroblast proliferation GO:0048146 10.12 PDGFRB PDGFRA ABL1
10 positive regulation of MAPK cascade GO:0043410 10.11 PDGFRB PDGFRA KIT JAK2 FGFR1
11 phosphatidylinositol-mediated signaling GO:0048015 10.1 FGFR1 PDGFRA PDGFRB
12 positive regulation of tyrosine phosphorylation of STAT protein GO:0042531 10.1 CSF2 IL3 JAK2 KIT
13 positive regulation of receptor signaling pathway via JAK-STAT GO:0046427 10.09 KIT JAK2 IL5
14 chemotaxis GO:0006935 10.08 RNASE3 PDGFRB PDGFRA CXCL8 CCL11
15 phosphorylation GO:0016310 10.07 ABL1 FGFR1 JAK2 KIT PDGFRA PDGFRB
16 positive regulation of phosphatidylinositol 3-kinase activity GO:0043552 10.06 PDGFRB PDGFRA KIT
17 immune response GO:0006955 10.06 CCL11 CSF2 CXCL8 IGHE IL2RA IL3
18 positive regulation of phosphatidylinositol 3-kinase signaling GO:0014068 9.96 PDGFRB PDGFRA KIT JAK2 FGFR1
19 response to fluid shear stress GO:0034405 9.95 PDGFRB CSF2
20 positive regulation of leukocyte proliferation GO:0070665 9.93 JAK2 CSF2
21 platelet-derived growth factor receptor-beta signaling pathway GO:0035791 9.92 PDGFRB ABL1
22 granulocyte-macrophage colony-stimulating factor signaling pathway GO:0038157 9.91 JAK2 CSF2
23 mast cell chemotaxis GO:0002551 9.9 KIT CCL11
24 metanephric glomerular capillary formation GO:0072277 9.88 PDGFRB PDGFRA
25 positive regulation of cell proliferation by VEGF-activated platelet derived growth factor receptor signaling pathway GO:0038091 9.87 PDGFRB PDGFRA
26 platelet-derived growth factor receptor signaling pathway GO:0048008 9.87 PDGFRB PDGFRA JAK2 ABL1
27 defense response to nematode GO:0002215 9.86 PRG2 EPX
28 multicellular organism development GO:0007275 9.83 PDGFRB PDGFRA KIT FGFR1
29 positive regulation of protein modification process GO:0031401 9.64 PDGFRB KIT
30 positive regulation of phospholipase C activity GO:0010863 9.56 PDGFRB PDGFRA KIT FGFR1
31 peptidyl-tyrosine phosphorylation GO:0018108 9.47 PDGFRB PDGFRA KIT JAK2 IL5 FGFR1

Molecular functions related to Hypereosinophilic Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 protein kinase activity GO:0004672 9.91 PDGFRB PDGFRA KIT JAK2 FGFR1 ABL1
2 SH2 domain binding GO:0042169 9.88 KIT JAK2 ABL1
3 kinase activity GO:0016301 9.87 PDGFRB PDGFRA KIT JAK2 FGFR1 ABL1
4 transmembrane receptor protein tyrosine kinase activity GO:0004714 9.76 FGFR1 KIT PDGFRA PDGFRB
5 vascular endothelial growth factor binding GO:0038085 9.67 PDGFRB PDGFRA
6 cytokine activity GO:0005125 9.65 IL5 IL3 IFNA1 CXCL8 CSF2 CCL11
7 protein tyrosine kinase activity GO:0004713 9.36 PDGFRB PDGFRA KIT JAK2 FGFR1 ABL1

Sources for Hypereosinophilic Syndrome

2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 08-Dec-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
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