HES
MCID: HYP098
MIFTS: 67

Hypereosinophilic Syndrome (HES)

Categories: Blood diseases, Immune diseases, Rare diseases

Aliases & Classifications for Hypereosinophilic Syndrome

MalaCards integrated aliases for Hypereosinophilic Syndrome:

Name: Hypereosinophilic Syndrome 12 75 53 59 37 55 15 17 72
Eosinophilia 12 75 29 55 44 15 72 33
Hes 53 59 48
Disseminated Eosinophilic Collagen Disease 72
Hypereosinophilic Syndrome, Idiopathic 53
Idiopathic Hypereosinophilic Syndrome 72
Eosinophilic Leukocytosis 12
Eosinophilic Disorder 72

Characteristics:

Orphanet epidemiological data:

59
hypereosinophilic syndrome
Inheritance: Not applicable; Prevalence: <1/1000000 (United States),1-9/100000 (Europe),1-9/100000 (United States); Age of onset: All ages; Age of death: any age;

Classifications:



External Ids:

Disease Ontology 12 DOID:999
KEGG 37 H01599
ICD9CM 35 288.3
MeSH 44 D004802
SNOMED-CT 68 27955006
ICD10 33 D72.1
MESH via Orphanet 45 D017681
ICD10 via Orphanet 34 D47.5
UMLS via Orphanet 73 C1540912
Orphanet 59 ORPHA168956
UMLS 72 C0014457 C0206141 C0263662 more

Summaries for Hypereosinophilic Syndrome

KEGG : 37
Hypereosinophilic syndrome (HES) is a heterogeneous group of rare disorders characterized by marked blood or tissue eosinophilia resulting in a wide variety of clinical manifestations. A number of HES subtypes, or variants, have recently been identified. The best characterized of the clonal molecular defects implicated in myeloproliferative variant HES is the 800-kb interstitial deletion on chromosome 4q12 resulting in the fusion of 2 distinct genes, Fip1-like 1 (FIP1L1) and platelet-derived growth factor receptor-a (PDGFRA), leading to the FIP1L1-PDGFRA fusion kinase. PDGFR is a membrane-bound tyrosine kinase receptor and the fusion PDGFRA kinase has constitutive/unregulated tyrosine kinase activity. The lymphocytic HES variant is associated with T-cell clones producing interleukin-5 (IL-5) and can evolve into lymphoma. While myeloproliferative and lymphocytic HES are well established and permit elimination of the term, idiopathic, to these varieties, most HES patients do not fall into these categories and are classified as complex.

MalaCards based summary : Hypereosinophilic Syndrome, also known as eosinophilia, is related to primary hypereosinophilic syndrome and myeloid and lymphoid neoplasms with eosinophilia and abnormalities of pdgfra, pdgfrb, and fgfr1, and has symptoms including nausea and vomiting, constipation and angina pectoris. An important gene associated with Hypereosinophilic Syndrome is PDGFRA (Platelet Derived Growth Factor Receptor Alpha), and among its related pathways/superpathways are Innate Immune System and ERK Signaling. The drugs Vancomycin and Nitric Oxide have been mentioned in the context of this disorder. Affiliated tissues include lung, t cells and myeloid, and related phenotypes are Decreased viability and Decreased viability

NIH Rare Diseases : 53 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. Management varies based on the severity of the condition and whether or not an underlying cause has been identified but generally includes imatinib or corticosteroids as an initial treatment.

Wikipedia : 75 Eosinophilia is a condition in which the eosinophil count in the peripheral blood exceeds 5.0?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 1409)
# Related Disease Score Top Affiliating Genes
1 primary hypereosinophilic syndrome 34.4 PDGFRB PDGFRA FIP1L1 FGFR1
2 myeloid and lymphoid neoplasms with eosinophilia and abnormalities of pdgfra, pdgfrb, and fgfr1 34.3 PDGFRB PDGFRA FGFR1
3 hypereosinophilic syndrome, idiopathic 34.2 PDGFRB PDGFRA KIT IL5 IL3 FIP1L1
4 pdgfra-associated chronic eosinophilic leukemia 33.2 PDGFRA FIP1L1
5 endomyocardial fibrosis 32.9 PDGFRA IL4 FIP1L1
6 8p11 myeloproliferative syndrome 32.7 PDGFRB KIT FGFR1
7 omenn syndrome 32.6 IL5 IL4 IGHE
8 polycythemia vera 31.9 PDGFRB PDGFRA KIT IL3 IFNA1 ABL1
9 paragonimiasis 31.6 RNASE3 IL5 IL13 CCL17
10 eosinophilic colitis 31.3 RNASE3 PRG2
11 intestinal schistosomiasis 31.2 IL5 IL4 IL13
12 chronic eosinophilic leukemia 31.1 PDGFRB PDGFRA KIT IFNA1 FIP1L1 FGFR1
13 churg-strauss syndrome 31.0 RNASE3 IL5 CCL17
14 wells syndrome 30.9 RNASE3 IL5 IL2RA
15 loeffler endocarditis 30.7 RNASE3 PDGFRA IL5 FIP1L1
16 chronic eosinophilic pneumonia 30.6 RNASE3 IL5 CCL11
17 systemic mastocytosis 30.5 PDGFRB PDGFRA KIT FIP1L1 FGFR1
18 eosinophilic gastroenteritis 30.5 RNASE3 IL5 IL3 EPX CCL11
19 kimura disease 30.4 RNASE3 IL5 IL4 CCL11
20 bullous pemphigoid 30.4 RNASE3 IL5 CCL11
21 pulmonary eosinophilia 30.4 RNASE3 IL5 IL4 IL13 EPX CCL17
22 eosinophilic granuloma 30.4 RNASE3 EPX
23 strongyloidiasis 30.4 IL5 IL13 CCL11
24 gastroenteritis 30.3 RNASE3 IL5 IL4 IFNA1 CCL11
25 myeloproliferative neoplasm 30.3 PDGFRB PDGFRA KIT FIP1L1 FGFR1 ABL1
26 milk allergy 30.3 RNASE3 IL5 IL4
27 atypical chronic myeloid leukemia 30.2 PDGFRB PDGFRA ABL1
28 leiomyosarcoma 30.2 PDGFRB PDGFRA KIT
29 sm-ahnmd 30.2 PDGFRA KIT FIP1L1
30 bronchitis 30.2 RNASE3 IL5 CCL11
31 pemphigoid gestationis 30.1 IL5 CCL11
32 desmoid tumor 30.1 PDGFRB PDGFRA KIT
33 esophagitis 30.0 IL5 IL13 CCL11
34 hairy cell leukemia 30.0 IL3 IL2RA IFNA1
35 contact dermatitis 29.9 IL5 IL4 CCL17
36 mycosis fungoides 29.9 IL5 IL2RA IFNA1 CCL17
37 dermatofibrosarcoma protuberans 29.9 PDGFRB PDGFRA KIT
38 esophagitis, eosinophilic, 1 29.9 IL5 IL13 CCL11
39 allergic contact dermatitis 29.8 IL5 IL4 CCL17
40 stevens-johnson syndrome/toxic epidermal necrolysis 29.8 IL5 IL13 CCL17
41 cytokine deficiency 29.8 IL5 IL13
42 intrinsic asthma 29.8 RNASE3 IL5 IL4 IL3
43 myeloid leukemia 29.8 KIT IL3 CSF2 ABL1
44 acquired immunodeficiency syndrome 29.8 IL2RA IFNA1 CSF2
45 cough variant asthma 29.7 RNASE3 IL5 IL4
46 gastrointestinal stromal tumor 29.7 PDGFRB PDGFRA KIT ABL1
47 folliculitis 29.7 IL5 IL4 IL2RA CCL11
48 otitis media 29.7 RNASE3 IL4 IGHE CCL11
49 blepharoconjunctivitis 29.6 RNASE3 IL4 CCL11
50 cutaneous t cell lymphoma 29.5 IL4 IL2RA IFNA1 CCL17

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, edema, pruritus, myalgia, chest pain, diarrhea, snoring, icterus, coughing, dyspepsia, heartburn, gastrointestinal gas

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

26 (show all 13)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased viability GR00173-A 9.68 PDGFRA
2 Decreased viability GR00221-A-1 9.68 ABL1 FGFR1 KIT PDGFRA PDGFRB
3 Decreased viability GR00221-A-2 9.68 ABL1 FGFR1
4 Decreased viability GR00221-A-3 9.68 ABL1 PDGFRA PDGFRB
5 Decreased viability GR00221-A-4 9.68 PDGFRA PDGFRB
6 Decreased viability GR00301-A 9.68 KIT
7 Decreased viability GR00342-S-1 9.68 ABL1 PDGFRB
8 Decreased viability GR00342-S-2 9.68 ABL1
9 Decreased viability GR00342-S-3 9.68 ABL1
10 Decreased viability GR00402-S-2 9.68 ABL1 FGFR1 KIT PDGFRA PDGFRB
11 Decreased substrate adherent cell growth GR00193-A-1 9.43 KIT
12 Decreased substrate adherent cell growth GR00193-A-2 9.43 ABL1 KIT
13 Decreased substrate adherent cell growth GR00193-A-4 9.43 ABL1 FGFR1 KIT

MGI Mouse Phenotypes related to Hypereosinophilic Syndrome:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 10 ABL1 CCL11 CCL17 CSF2 EPX FGFR1
2 digestive/alimentary MP:0005381 9.97 ABL1 FGFR1 IL13 IL2RA IL4 IL5
3 craniofacial MP:0005382 9.91 ABL1 CSF2 FGFR1 IL4 KIT PDGFRA
4 endocrine/exocrine gland MP:0005379 9.91 ABL1 CSF2 FGFR1 IL13 IL2RA IL4
5 immune system MP:0005387 9.8 ABL1 CCL11 CCL17 CSF2 EPX FGFR1
6 respiratory system MP:0005388 9.32 ABL1 CCL11 CSF2 IL13 IL2RA IL4

Drugs & Therapeutics for Hypereosinophilic Syndrome

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

(show top 50) (show all 300)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Vancomycin Approved Phase 4 1404-90-6 441141 14969
2
Nitric Oxide Approved Phase 4 10102-43-9 145068
3
Ciclesonide Approved, Investigational Phase 4 126544-47-6, 141845-82-1 444033
4
Omalizumab Approved, Investigational Phase 4 242138-07-4
5
Diethylcarbamazine Approved, Investigational, Vet_approved Phase 4 90-89-1 3052
6
Cefpirome Approved Phase 4 84957-29-9 5479539
7
Moxifloxacin Approved, Investigational Phase 4 354812-41-2, 151096-09-2 152946
8
Norgestimate Approved, Investigational Phase 4 35189-28-7 6540478
9
Ethinyl Estradiol Approved Phase 4 57-63-6 5991
10
Estradiol Approved, Investigational, Vet_approved Phase 4 50-28-2 5757
11
Polyestradiol phosphate Approved Phase 4 28014-46-2
12
Stavudine Approved, Investigational Phase 4 3056-17-5 18283
13
Lamivudine Approved, Investigational Phase 4 134678-17-4 60825
14
Nevirapine Approved Phase 4 129618-40-2 4463
15
Efavirenz Approved, Investigational Phase 4 154598-52-4 64139
16
Ivermectin Approved, Investigational, Vet_approved Phase 4 70288-86-7 6474909
17
Sotalol Approved Phase 4 959-24-0, 3930-20-9 5253
18 Benralizumab Approved, Investigational Phase 4 1044511-01-4
19
Dexlansoprazole Approved, Investigational Phase 4 103577-45-3, 138530-94-6 9578005
20
Lansoprazole Approved, Investigational Phase 4 103577-45-3 3883
21 Sympathomimetics Phase 4
22 Fluticasone-Salmeterol Drug Combination Phase 4
23 Parasympatholytics Phase 4
24 Muscarinic Antagonists Phase 4
25 Tiotropium Bromide Phase 4 136310-93-5
26 Antimetabolites Phase 4
27 Topoisomerase Inhibitors Phase 4
28 Nucleic Acid Synthesis Inhibitors Phase 4
29 Reverse Transcriptase Inhibitors Phase 4
30 Adrenergic Agonists Phase 4
31 Adrenergic beta-2 Receptor Agonists Phase 4
32 Adrenergic beta-Agonists Phase 4
33
Salmeterol xinafoate Phase 4 94749-08-3 56801
34 Immunoglobulin E Phase 4
35 Lipoxygenase Inhibitors Phase 4
36 Albuterol Phase 4
37 Tocolytic Agents Phase 4
38 Cytochrome P-450 Enzyme Inhibitors Phase 4
39 Contraceptives, Oral Phase 4
40 Contraceptive Agents Phase 4
41 Contraceptives, Oral, Combined Phase 4
42 Norgestimate, ethinyl estradiol drug combination Phase 4
43 Cephalosporins Phase 4
44 Estradiol 3-benzoate Phase 4
45 Estradiol 17 beta-cypionate Phase 4
46 Cytochrome P-450 CYP2C9 Inhibitors Phase 4
47 Cytochrome P-450 CYP2C19 Inhibitors Phase 4
48 Cytochrome P-450 CYP3A Inducers Phase 4
49 Neurotransmitter Agents Phase 4
50 Peripheral Nervous System Agents Phase 4

Interventional clinical trials:

(show top 50) (show all 264)
# Name Status NCT ID Phase Drugs
1 Randomized Double-blind Placebo-controlled Study of add-on Montelukast to Inhaled Budesonide in the Treatment of Nonasthmatic Eosinophilic Bronchitis Unknown status NCT01121016 Phase 4 Montelukast
2 Evaluation of Effectiveness and Safety of Oral Vancomycin in Treatment of Primary Sclerosing Cholangitis. Unknown status NCT02605213 Phase 4 Vancomycin;Placebo
3 The Treatment Effect of Inhaled Corticosteroid and Long-acting beta2 Agonist Combination Versus Long-acting Anti-cholinergic Agent on Stratified COPD Patients Based on the Levels of Exhaled Nitric Oxide Unknown status NCT02546349 Phase 4 fluticasone/salmeterol, tiotropium
4 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
5 Small Particle Inhaled Steroids in Refractory Steroid-responsive Asthma Completed NCT01171365 Phase 4 Ciclesonide;Placebo
6 Efficacy and Safety of Lectranal® in Treatment of Seasonal Allergic Rhinitis Symptoms Completed NCT00460538 Phase 4
7 The Effects of Montelukast on Sputum Cells and Inflammatory Markers in Smokers With Asthma Completed NCT00712335 Phase 4 Fluticasone Propionate;Montelukast;Salmeterol
8 A Randomized, Multi-center, Double-blind, Placebo-controlled, Parallel-group Trial to Explore the Effects of 78 Weeks Omalizumab Treatment Given as Add on Therapy on Markers of Airway Inflammation and Remodeling in Patients With Moderate to Severe Persistent Allergic Asthma Receiving Inhaled Corticosteroids and Long Acting Beta-agonists Completed NCT00670930 Phase 4 omalizumab at a dose of 0.016mg/kg/IU/mL;Placebo
9 A Description of Inflammatory Cell Types in Moderate to Severe Pediatric Asthma: Eosinophilic and Non Eosinophilic Sputum Markers While on Anti-IgE Therapy (Xolair) Completed NCT00283504 Phase 4 ANTI-IGE THERAPY (XOLAIR)
10 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
11 Plasma and Abscess Fluid Pharmacokinetics of Cefpirome and Moxifloxacin After Single and Multiple Dose Administration Completed NCT00280514 Phase 4 cefpirome and moxifloxacin administration
12 A Retrospective Study to Compare the 3-Year Antiviral Efficacy of Nevirapine and Efavirenz in Combination With D4t and 3tc in 2NN Patients and of Trizivir Versus Trizivir Plus Nevirapine in CHARM Patients Completed NCT00127972 Phase 4 stavudine;lamivudine;nevirapine;efavirenz;trizivir
13 Comparison Between the Post-Treatment Reactions After Single-dose Ivermectin or DEC in Subjects With Loa Loa Infection Completed NCT01593722 Phase 4 Diethylcarbamazine;Ivermectin
14 Assessment of Glucocorticoid Responsiveness and Mechanisms of Resistance in Hypereosinophilic Syndromes Recruiting NCT01524536 Phase 4 prednisone
15 A Pilot and Feasibility Study to Determine if a Common Atrial Fibrillation Risk Locus Modulates Differential Response to Antiarrhythmic Drugs Recruiting NCT02347111 Phase 4 Flecainide;Sotalol
16 Impact of Benralizumab Treatment on Circulating Dendritic Cells in Patients With Eosinophilic Asthma Not yet recruiting NCT03652376 Phase 4 Benralizumab
17 The Frequency of Eosinophilic Esophagitis in Patients With Heartburn That is Refractory to Proton Pump Inhibitors Terminated NCT01404832 Phase 4 Treat with lansoprazole 30 mg BID for 2 weeks, endoscopic examination with esophageal biopsy for patients with persistent symptoms
18 Effect of Concomitant Mansonella Perstans Microfilaremia on Immune Responses Following Single Dose Praziquantel in Subjects With Schistosomiasis: A Pilot Study Withdrawn NCT02734186 Phase 4 Praziquantel
19 Treatment of Asymptomatic Toxocariasis With Albendazole in Children. A Prospective, Randomized, Placebo-controlled Clinical Trial Unknown status NCT00755560 Phase 3 Albendazole;Placebo
20 Prednisone in Chronic Rhinosinusitis Without Nasal Polyps. A Double-blind, Randomized, Placebo-controlled Trial Unknown status NCT02367118 Phase 3 Prednisone;Placebo
21 The Efficacy of Mepolizumab Treatment on Rhinovirus Induced Asthma Exacerbations Unknown status NCT01520051 Phase 3 Mepolizumab;Placebo
22 Vitamin D Supplementation as an Adjuvant Therapy for Specific Immunotherapy in Patients With Allergic Rhinitis. Unknown status NCT02738619 Phase 2, Phase 3 Vitamin D3;placebo
23 INSPIRE: An Internet-based RCT for Long-term Survivors of Hematopoietic Stem Cell Transplantation Completed NCT00799461 Phase 3
24 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
25 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
26 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
27 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
28 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
29 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
30 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
31 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 &lt; 60 Years Completed NCT00006363 Phase 3 cytarabine;daunorubicin hydrochloride;etoposide;valspodar;busulfan
32 Route of Administration of Anti-IL5 Monoclonal Antibody in Prednisone-dependent Eosinophilic Asthma Completed NCT02559791 Phase 2, Phase 3 Placebo
33 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
34 Mepolizumab in COPD With Eosinophilic Bronchitis: A Randomized Clinical Trial Completed NCT01463644 Phase 3 Mepolizumab;placebo
35 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)
36 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
37 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
38 GPIAG and Leicester Asthma and Dysfunctional Breathing (GLAD) Study: a Randomised Controlled Study Completed NCT00515840 Phase 3
39 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
40 Particulate Reduction Education in City Homes (PREACH) Completed NCT00466024 Phase 3
41 A Double-blind Placebo Controlled Clinical Trial to Evaluate the Effects on Asthma Control of Rabeprazole Given Twice Daily in Subjects With Asthma. Completed NCT00214552 Phase 3 rabeprazole
42 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
43 The Use of Inhaled Corticosteroids in the Treatment of Asthma is Children in the Emergency Room Completed NCT01524198 Phase 2, Phase 3 Budesonide;Normal saline
44 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 Recruiting NCT03306043 Phase 3 Mepolizumab
45 Benralizumab in Patients With Inadequate Response to Anti-IL5 Monoclonal Antibody Therapies Recruiting NCT03470311 Phase 3
46 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
47 Assessment of the Impact of a Dietary Supplement Synbiotic "Probiotical ®" in Asthma Recruiting NCT03341403 Phase 2, Phase 3
48 Maraviroc to Augment Rehabilitation Outcomes After Stroke Recruiting NCT03172026 Phase 2, Phase 3 Maraviroc 300 mg;Placebo 300 mg
49 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 Active, not recruiting NCT02836496 Phase 3 Mepolizumab 300 mg;Placebo matching mepolizumab;Active OCS capsules (5 mg prednisolone or prednisone);Placebo matching OCS capsules
50 Mepolizumab Long-term Access Programme for Subjects Who Participated in Study MEA115921 (Placebo-controlled Study of Mepolizumab in the Treatment of Eosinophilic Granulomatosis With Polyangiitis in Subjects Receiving Standard-of-care Therapy) Active, not recruiting NCT03298061 Phase 3 Mepolizumab;Prednisolone

Search NIH Clinical Center for Hypereosinophilic Syndrome

Inferred drug relations via UMLS 72 / NDF-RT 51 :


hydroxyurea
Hydroxyurea
imatinib
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 29

Anatomical Context for Hypereosinophilic Syndrome

MalaCards organs/tissues related to Hypereosinophilic Syndrome:

41
Lung, T Cells, Myeloid, Skin, Bone, Testes, Liver

Publications for Hypereosinophilic Syndrome

Articles related to Hypereosinophilic Syndrome:

(show top 50) (show all 20282)
# Title Authors PMID Year
1
Early phase resolution of mucosal eosinophilic inflammation in allergic rhinitis. 9 38
20459697 2010
2
Delayed-type asthmatic response to bronchial challenge with allergen, I: clinical features. 9 38
20486329 2010
3
Allergen-induced, eotaxin-rich, proangiogenic bone marrow progenitors: a blood-borne cellular envoy for lung eosinophilia. 9 38
20227754 2010
4
Thrombotic storm in Kimura disease. 9 38
19468829 2010
5
Airway wall expression of OX40/OX40L and interleukin-4 in asthma. 9 38
20139223 2010
6
Critical role of the platelet-derived growth factor receptor (PDGFR) beta transmembrane domain in the TEL-PDGFRbeta cytosolic oncoprotein. 9 38
20164181 2010
7
IL-13 Gene Polymorphisms are Associated With Rhinosinusitis and Eosinophilic Inflammation in Aspirin Intolerant Asthma. 9 38
20358028 2010
8
Hypereosinophilic syndrome and clonal eosinophilia: point-of-care diagnostic algorithm and treatment update. 9 38
20053713 2010
9
Vernal keratoconjunctivitis--a rare but serious comorbidity of allergic rhinitis and eustachian tube dysfunction. 9 38
19900723 2010
10
Hyper-IgE syndrome with STAT3 mutation: a case report in Mainland China. 9 38
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. 9 38
19864487 2010
12
Effects of omalizumab on markers of inflammation in patients with allergic asthma. 9 38
19839977 2009
13
A diagnostically difficult case of chronic myeloid neoplasm with eosinophilia and abnormalities of PDGFRA effectively treated with imatinib in accelerated phase: case report. 9 38
20010473 2009
14
Concomitant occurrence of kimura disease and mycosis fungoides in a Lebanese woman: significance and response to rituximab. 9 38
19786854 2009
15
Kimura's disease presenting with a giant suspensory tumor and associated with membranoproliferative glomerulonephritis. 9 38
19620037 2009
16
Triptolide abrogates oncogene FIP1L1-PDGFRalpha addiction and induces apoptosis in hypereosinophilic syndrome. 9 38
19671059 2009
17
Myeloproliferative neoplasms: contemporary diagnosis using histology and genetics. 9 38
19806146 2009
18
A low dose of Mycoplasma pneumoniae infection enhances an established allergic inflammation in mice: the role of the prostaglandin E2 pathway. 9 38
19552640 2009
19
Is serum total IgE levels a good predictor of allergies in children? 9 38
19813686 2009
20
Recurrent giant chalazia in hyperimmunoglobulin E (Job's) syndrome. 9 38
18528638 2009
21
Leukotriene E4-induced pulmonary inflammation is mediated by the P2Y12 receptor. 9 38
19822647 2009
22
Innate cells and T helper 2 cell immunity in airway inflammation. 9 38
19766085 2009
23
Recent breakthroughs in the understanding and management of chronic eosinophilic leukemia. 9 38
19761433 2009
24
T-helper type 2-driven inflammation defines major subphenotypes of asthma. 9 38
19483109 2009
25
Changed concepts and definitions of myeloproliferative neoplasms (MPN), myelodysplastic syndromes (MDS) and myelodysplastic/myeloproliferative neoplasms (MDS/MPN) in the updated 2008 WHO classification. 9 38
20309429 2009
26
Similar colds in subjects with allergic asthma and nonatopic subjects after inoculation with rhinovirus-16. 9 38
19596142 2009
27
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. 9 38
19386785 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. 9 38
19630858 2009
29
Clinical significance of eosinophilic cationic protein levels in nasal secretions of patients with nasal polyposis. 9 38
19034473 2009
30
Production of interleukin-10 in asthmatic children after Beta-1-3-glucan. 9 38
19912977 2009
31
Imatinib has limited therapeutic activity for hypereosinophilic syndrome patients with unknown or negative PDGFRalpha mutation status. 9 38
19013640 2009
32
A novel FIP1L1-PDGFRA mutant destabilizing the inactive conformation of the kinase domain in chronic eosinophilic leukemia/hypereosinophilic syndrome. 9 38
19210352 2009
33
Pathogenesis of Churg-Strauss syndrome: recent insights. 9 38
19811306 2009
34
Eosinophilia-associated muscle disorders: an immunohistological study with tissue localisation of major basic protein in distinct clinicopathological forms. 9 38
19139036 2009
35
A scleroderma-like cutaneous syndrome associated with a marked Th2-type immune response occurring after a prosthetic joint implant. 9 38
19300283 2009
36
IL-25: a key requirement for the regulation of type-2 immunity. 9 38
19449446 2009
37
VCAM-1 and eosinophilia in diffuse sino-nasal polyps. 9 38
18642014 2009
38
Reciprocal regulation of iNOS and PARP-1 during allergen-induced eosinophilia. 9 38
18829681 2009
39
Molecular drug targets in myeloproliferative neoplasms: mutant ABL1, JAK2, MPL, KIT, PDGFRA, PDGFRB and FGFR1. 9 38
19175693 2009
40
Evidence that 13-14 di-hydro, 15-keto prostaglandin D(2)-induced airway eosinophilia in guinea-pigs is independent of interleukin-5. 9 38
19184361 2009
41
Sputum IL-5 concentration is associated with a sputum eosinophilia and attenuated by corticosteroid therapy in COPD. 9 38
19478474 2009
42
Th2 cells as targets for therapeutic intervention in allergic bronchial asthma. 9 38
19099351 2009
43
Kimura's disease in a young Balkan male. 9 38
19195268 2009
44
Thrombosis of temporal artery and renal vein in Kimura-disease-related nephrotic syndrome. 9 38
18066701 2009
45
Immunosuppression of TH2 responses in Trichinella spiralis infection by Helicobacter pylori neutrophil-activating protein. 9 38
18804852 2008
46
Five years since the discovery of FIP1L1-PDGFRA: what we have learned about the fusion and other molecularly defined eosinophilias. 9 38
18843283 2008
47
TARC and IL-5 expression correlates with tissue eosinophilia in peripheral T-cell lymphomas. 9 38
18395252 2008
48
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. 9 38
18699933 2008
49
CD4+CD25+ regulatory T cells reverse established allergic airway inflammation and prevent airway remodeling. 9 38
18672278 2008
50
Liver X receptor agonists increase airway reactivity in a model of asthma via increasing airway smooth muscle growth. 9 38
18768884 2008

Variations for Hypereosinophilic Syndrome

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 54)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.98 RNASE3 PRG2 PDGFRB PDGFRA KIT IL5
2
Show member pathways
13.89 PRG2 PDGFRB PDGFRA KIT IL5 IL4
3
Show member pathways
13.71 PDGFRB PDGFRA KIT IL5 IL4 IL3
4
Show member pathways
13.58 PDGFRB PDGFRA KIT IL5 IL4 IL3
5
Show member pathways
13.53 PDGFRB PDGFRA KIT IL5 IL3 IL2RA
6
Show member pathways
13.42 PDGFRB PDGFRA KIT IL5 IL4 IL3
7
Show member pathways
13.29 PDGFRB PDGFRA KIT IL5 IL4 IL3
8
Show member pathways
13.28 PDGFRB PDGFRA KIT IL5 IL4 IL3
9
Show member pathways
13.1 PRG2 PDGFRB PDGFRA KIT IL3 IL2RA
10
Show member pathways
13.08 PDGFRB PDGFRA KIT IL4 IL3 IL2RA
11
Show member pathways
13.06 PDGFRB PDGFRA KIT IL4 IL3 IL2RA
12
Show member pathways
12.93 PDGFRB PDGFRA KIT IL3 FGFR1 CSF2
13 12.79 PDGFRB PDGFRA KIT IL5 IL4 IL3
14
Show member pathways
12.74 PDGFRB PDGFRA KIT FGFR1 ABL1
15
Show member pathways
12.67 RNASE3 PRG2 PDGFRA IL5 IL4 IL3
16
Show member pathways
12.65 IL5 IL4 IL3 IL13 IGHE CSF2
17 12.59 KIT IL4 IL3 IL2RA IFNA1
18
Show member pathways
12.53 PDGFRB PDGFRA IL5 IL2RA IL13 FGFR1
19
Show member pathways
12.49 IL4 IL3 IFNA1 CSF2
20
Show member pathways
12.43 IL5 IL4 IL13 CSF2 CCL17 CCL11
21
Show member pathways
12.4 PDGFRB PDGFRA KIT IL3 FGFR1
22 12.35 PDGFRB PDGFRA IL2RA CSF2
23
Show member pathways
12.32 PDGFRB PDGFRA IL5 IL4 IL3 IL2RA
24
Show member pathways
12.2 PDGFRB PDGFRA IL2RA FGFR1
25 12.2 PDGFRB PDGFRA KIT FGFR1 ABL1
26
Show member pathways
12.17 IL5 IL3 IL2RA CSF2
27 12.15 PDGFRB PDGFRA IL2RA FGFR1
28
Show member pathways
12.15 IL5 IL4 IL3 IL2RA IL13 FGFR1
29 12.09 KIT IL5 IL4 IL3 IL2RA CSF2
30 12.03 IL4 IL13 IGHE CCL11
31 11.99 KIT IL5 IL4 IL3 IL2RA CSF2
32 11.92 IL3 IL2RA IFNA1 ABL1
33 11.91 KIT IL5 IL4 IL2RA IL13
34
Show member pathways
11.88 IL5 IL4 IL3 IL2RA CSF2
35 11.87 IL5 IL4 IL13 CSF2
36 11.85 IL4 IL13 CSF2 CCL17 CCL11
37 11.84 PDGFRB PDGFRA FGFR1
38
Show member pathways
11.8 PDGFRB KIT FGFR1
39 11.79 PDGFRB PDGFRA KIT FGFR1
40 11.79 IL5 IL4 IL13 CSF2 CCL11
41 11.78 IL5 IL4 IGHE CCL17 CCL11
42
Show member pathways
11.57 PDGFRB PDGFRA KIT IL5 IL4 IL3
43 11.56 IL4 IL13 CSF2
44 11.55 IL5 IL3 CSF2
45 11.54 IL5 IL4 IL2RA IL13 CCL17 CCL11
46 11.52 PDGFRB PDGFRA FGFR1
47 11.49 PDGFRB PDGFRA FGFR1
48 11.37 IL5 IL4 IL2RA
49 11.35 IL5 IL4 IL13
50 11.34 IL5 IL4 IL3 IL13 CSF2

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 9.7 RNASE3 KIT IL5 IL4 IL3 IL13
2 receptor complex GO:0043235 9.56 PDGFRB PDGFRA KIT FGFR1
3 external side of plasma membrane GO:0009897 9.55 PDGFRA KIT IL2RA IL13 IGHE
4 extracellular region GO:0005576 9.44 RNASE3 PRG2 IL5 IL4 IL3 IL13

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

(show all 38)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of cell proliferation GO:0008284 9.98 PDGFRB PDGFRA KIT IL5 IL3 FGFR1
2 defense response to bacterium GO:0042742 9.95 RNASE3 PRG2 IGHE EPX
3 positive regulation of cell migration GO:0030335 9.95 PDGFRB PDGFRA KIT CCL11
4 regulation of cell proliferation GO:0042127 9.93 KIT FGFR1 CSF2 ABL1
5 positive regulation of protein kinase B signaling GO:0051897 9.92 PDGFRB PDGFRA KIT FGFR1
6 chemotaxis GO:0006935 9.9 PDGFRB PDGFRA CCL17 CCL11
7 positive regulation of MAPK cascade GO:0043410 9.87 PDGFRB PDGFRA KIT FGFR1
8 MAPK cascade GO:0000165 9.86 PDGFRB PDGFRA KIT IL5 IL3 IL2RA
9 transmembrane receptor protein tyrosine kinase signaling pathway GO:0007169 9.85 PDGFRB PDGFRA KIT
10 protein autophosphorylation GO:0046777 9.85 PDGFRB PDGFRA KIT FGFR1 ABL1
11 positive regulation of ERK1 and ERK2 cascade GO:0070374 9.85 PDGFRB PDGFRA KIT CCL17 CCL11 ABL1
12 immune response GO:0006955 9.85 PRG2 IL5 IL4 IL3 IL2RA IL13
13 positive regulation of peptidyl-tyrosine phosphorylation GO:0050731 9.84 IL5 IL3 ABL1
14 cell chemotaxis GO:0060326 9.84 PDGFRB PDGFRA KIT
15 positive regulation of MAP kinase activity GO:0043406 9.81 PDGFRB KIT FGFR1
16 positive regulation of tyrosine phosphorylation of STAT protein GO:0042531 9.8 KIT IL4 IL3 IL13 CSF2
17 positive regulation of phosphatidylinositol 3-kinase signaling GO:0014068 9.78 PDGFRB PDGFRA KIT FGFR1
18 phosphatidylinositol-mediated signaling GO:0048015 9.77 PDGFRB PDGFRA FGFR1
19 positive regulation of B cell proliferation GO:0030890 9.75 IL5 IL4 IL13
20 negative regulation of endothelial cell apoptotic process GO:2000352 9.73 IL4 IL13 ABL1
21 positive regulation of phosphatidylinositol 3-kinase activity GO:0043552 9.69 PDGFRB PDGFRA KIT
22 embryonic hemopoiesis GO:0035162 9.68 KIT IL3
23 negative regulation of apoptotic process GO:0043066 9.67 PDGFRB IL4
24 platelet-derived growth factor receptor signaling pathway GO:0048008 9.67 PDGFRB PDGFRA ABL1
25 positive regulation of podosome assembly GO:0071803 9.66 IL5 CSF2
26 dendritic cell differentiation GO:0097028 9.65 IL4 CSF2
27 response to fluid shear stress GO:0034405 9.64 PDGFRB CSF2
28 retina vasculature development in camera-type eye GO:0061298 9.64 PDGFRB PDGFRA
29 positive regulation of phospholipase C activity GO:0010863 9.62 PDGFRB PDGFRA KIT FGFR1
30 mast cell chemotaxis GO:0002551 9.61 KIT CCL11
31 platelet-derived growth factor receptor-beta signaling pathway GO:0035791 9.58 PDGFRB ABL1
32 positive regulation of cell proliferation by VEGF-activated platelet derived growth factor receptor signaling pathway GO:0038091 9.55 PDGFRB PDGFRA
33 negative regulation of complement-dependent cytotoxicity GO:1903660 9.54 IL4 IL13
34 metanephric glomerular capillary formation GO:0072277 9.52 PDGFRB PDGFRA
35 peptidyl-tyrosine phosphorylation GO:0018108 9.35 PDGFRB PDGFRA KIT FGFR1 ABL1
36 cytokine-mediated signaling pathway GO:0019221 9.32 KIT IL5 IL4 IL3 IL2RA IL13
37 protein phosphorylation GO:0006468 10.09 PDGFRB PDGFRA KIT FGFR1 CCL11 ABL1
38 inflammatory response GO:0006954 10.02 KIT IL5 IL13 CCL17 CCL11

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 9.83 PDGFRB PDGFRA KIT FGFR1 ABL1
2 growth factor activity GO:0008083 9.67 IL5 IL4 IL3 CSF2
3 cytokine activity GO:0005125 9.56 IL5 IL4 IL3 IL13 IFNA1 CSF2
4 platelet-derived growth factor receptor binding GO:0005161 9.46 PDGFRB PDGFRA
5 transmembrane receptor protein tyrosine kinase activity GO:0004714 9.46 PDGFRB PDGFRA KIT FGFR1
6 cytokine receptor binding GO:0005126 9.43 IL4 IL13 IFNA1
7 platelet-derived growth factor binding GO:0048407 9.4 PDGFRB PDGFRA
8 vascular endothelial growth factor binding GO:0038085 9.32 PDGFRB PDGFRA
9 protein tyrosine kinase activity GO:0004713 9.02 PDGFRB PDGFRA KIT FGFR1 ABL1

Sources for Hypereosinophilic Syndrome

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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