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HES
MCID: HYP098
MIFTS: 66

Hypereosinophilic Syndrome (HES)

Categories: Blood diseases, Cancer diseases, Immune diseases, Rare diseases
Genes Variations Tissues Related diseases Publications Pathways Symptoms & Phenotypes Drugs
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Aliases & Classifications for Hypereosinophilic Syndrome

About this section

MalaCards integrated aliases for Hypereosinophilic Syndrome:

Name: Hypereosinophilic Syndrome 12 73 20 58 36 54 15 17 70
Eosinophilia 12 73 29 54 6 44 15 70 32
Hes 20 58 48
Disseminated Eosinophilic Collagen Disease 70
Hypereosinophilic Syndrome, Idiopathic 20
Idiopathic Hypereosinophilic Syndrome 70
Eosinophilic Leukocytosis 12
Eosinophilic Disorder 70

Characteristics:

Orphanet epidemiological data:

58
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:

MalaCards categories:
Global: Rare diseases Cancer diseases
Anatomical: Immune diseases Blood diseases
See all MalaCards categories (disease lists)
ICD10: 32 33
Orphanet: 58  
Rare haematological diseases


External Ids:

Disease Ontology 12 DOID:999
KEGG 36 H01599
ICD9CM 34 288.3
MeSH 44 D004802
SNOMED-CT 67 418928000
ICD10 32 D72.1
MESH via Orphanet 45 D017681
ICD10 via Orphanet 33 D47.5
UMLS via Orphanet 71 C1540912
Orphanet 58 ORPHA168956
UMLS 70 C0014457 C0206141 C0263662 more
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Summaries for Hypereosinophilic Syndrome

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KEGG : 36 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 hypereosinophilic syndrome, idiopathic and myeloproliferative disorder, chronic, with eosinophilia, 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 Budesonide and Omalizumab have been mentioned in the context of this disorder. Affiliated tissues include myeloid, bone marrow and t cells, and related phenotypes are Decreased viability and Decreased viability

Disease Ontology : 12 A leukocyte disease that is characterized by high numbers of eosinophils which over time enter various tissues, eventually damaging organs.

GARD : 20 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 : 73 Eosinophilia is a condition in which the eosinophil count in the peripheral blood exceeds 5×108/L... more...

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Related Diseases for Hypereosinophilic Syndrome

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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 1318)
# Related Disease Score Top Affiliating Genes
1 hypereosinophilic syndrome, idiopathic 33.1 PDGFRB PDGFRA KIT IL5 IL3 FIP1L1
2 myeloproliferative disorder, chronic, with eosinophilia 32.8 PDGFRB FIP1L1
3 primary hypereosinophilic syndrome 32.6 PDGFRB PDGFRA FIP1L1 FGFR1
4 pdgfra-associated chronic eosinophilic leukemia 32.5 PDGFRA FIP1L1
5 loeffler syndrome 32.5 IL5 FIP1L1
6 myeloid and lymphoid neoplasms with eosinophilia and abnormalities of pdgfra, pdgfrb, and fgfr1 32.4 PDGFRB PDGFRA FGFR1
7 endomyocardial fibrosis 32.1 RNASE3 PRG2 PDGFRB PDGFRA IL5 FIP1L1
8 8p11 myeloproliferative syndrome 31.9 PDGFRB KIT FGFR1
9 paragonimiasis 31.9 RNASE3 IL5 IGHE
10 aggressive systemic mastocytosis 31.9 PDGFRB PDGFRA KIT IFNA1 FIP1L1
11 myeloid and lymphoid neoplasms associated with fgfr1 abnormalities 31.8 IL3 IL2RA FGFR1
12 pulmonary eosinophilia 31.7 RNASE3 PRG2 IL5 EPX CXCL8 CSF2
13 severe congenital neutropenia 31.6 JAK2 IL3 CXCL8 CSF2
14 polycythemia vera 31.5 PDGFRB PDGFRA KIT JAK2 IL3 IFNA1
15 myeloid and lymphoid neoplasms associated with pdgfra rearrangement 31.3 PDGFRB PDGFRA KIT JAK2 FIP1L1 FGFR1
16 chronic eosinophilic leukemia 31.2 PDGFRB PDGFRA KIT JAK2 IL5 IFNA1
17 strongyloidiasis 31.1 IL5 IGHE CCL11
18 eosinophilic colitis 31.0 RNASE3 PRG2
19 exanthem 31.0 KIT IFNA1 CXCL8 CSF2
20 churg-strauss syndrome 31.0 RNASE3 PRG2 IL5 IL3 CXCL8 CCL11
21 aspergillosis 30.9 IL5 EPX CXCL8 CSF2
22 budd-chiari syndrome 30.9 PDGFRA JAK2 FIP1L1
23 allergic bronchopulmonary aspergillosis 30.9 IL5 IGHE IGES EPX
24 wells syndrome 30.8 RNASE3 IL5 IL2RA CXCL8
25 pulmonary disease, chronic obstructive 30.8 RNASE3 IL5 CXCL8 CCL11
26 allergic rhinitis 30.8 RNASE3 IL5 IGHE IGES EPX CXCL8
27 toxocariasis 30.7 RNASE3 IL5 IGHE FIP1L1
28 eosinophilic pneumonia 30.7 RNASE3 IL5 IL3 CXCL8 CSF2 CCL11
29 chronic rhinitis 30.7 RNASE3 PRG2 IL5 EPX
30 trichinosis 30.6 IL5 IL3 CCL11
31 proteasome-associated autoinflammatory syndrome 1 30.6 RNASE3 IL5 IGHE IFNA1 CXCL8 CCL11
32 cellulitis 30.6 RNASE3 IL5 IGES CSF2
33 eosinophilic gastritis 30.6 IL5 FIP1L1 EPX CCL11
34 urticaria 30.6 RNASE3 PRG2 KIT IL5 IL3 IGHE
35 conjunctivitis 30.6 RNASE3 IL5 IGHE CXCL8 CCL11
36 eosinophilic gastroenteritis 30.6 RNASE3 PRG2 IL5 IL3 EPX CSF2
37 loeffler endocarditis 30.6 RNASE3 PRG2 PDGFRA IL5 IL3 FIP1L1
38 leukemia 30.6 KIT JAK2 IL3 FGFR1 CSF2 ABL1
39 respiratory failure 30.6 RNASE3 IL5 EPX CXCL8 CCL11
40 mastocytosis 30.5 PDGFRB PDGFRA KIT JAK2 IL3 IL2RA
41 myeloid leukemia 30.5 KIT JAK2 IL3 CSF2 ABL1
42 systemic mastocytosis 30.5 PDGFRB PDGFRA KIT JAK2 IL2RA IFNA1
43 food allergy 30.5 RNASE3 IL5 IGHE IGES EPX CXCL8
44 bronchiolitis 30.5 RNASE3 IL5 IFNA1 CXCL8 CSF2 CCL11
45 acute leukemia 30.5 KIT JAK2 IL3 FGFR1 CSF2
46 folliculitis 30.5 IL5 IL2RA CCL11
47 toxic oil syndrome 30.5 IL5 IL2RA IGHE
48 eosinophilic meningitis 30.5 IL5 IGHE CCL11
49 leukemia, acute lymphoblastic 30.5 KIT JAK2 IL3 FGFR1 CSF2 ABL1
50 milk allergy 30.5 RNASE3 IL5 IGHE IGES

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
Sources

Symptoms & Phenotypes for Hypereosinophilic Syndrome

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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:

26 (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 Decreased substrate adherent cell growth GR00193-A-1 9.63 KIT
14 Decreased substrate adherent cell growth GR00193-A-2 9.63 ABL1 KIT
15 Decreased substrate adherent cell growth GR00193-A-4 9.63 ABL1 FGFR1 KIT
16 Increased cell death in breast cancer cell lines (MCF10A, MDA-MB-435) GR00104-A-0 8.92 CSF2 CXCL8 IL2RA PDGFRB

MGI Mouse Phenotypes related to Hypereosinophilic Syndrome:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.97 ABL1 CCL11 CSF2 EPX FGFR1 IL2RA
2 immune system MP:0005387 9.77 ABL1 CCL11 CSF2 EPX FGFR1 IL2RA
3 respiratory system MP:0005388 9.28 ABL1 CCL11 CSF2 IL2RA IL5 JAK2
Sources

Drugs & Therapeutics for Hypereosinophilic Syndrome

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

(show top 50) (show all 185)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Budesonide Approved Phase 4 51333-22-3 63006 5281004
2
Omalizumab Approved, Investigational Phase 4 242138-07-4
3
Azithromycin Approved Phase 4 83905-01-5 55185 447043
4
Benralizumab Approved, Investigational Phase 4 1044511-01-4
5
Prednisone Approved, Vet_approved Phase 4 53-03-2 5865
6
Methylprednisolone Approved, Vet_approved Phase 4 83-43-2 6741
7
Methylprednisolone hemisuccinate Approved Phase 4 2921-57-5
8
Prednisolone Approved, Vet_approved Phase 4 50-24-8 5755
9
Prednisolone acetate Approved, Vet_approved Phase 4 52-21-1
10
Prednisolone phosphate Approved, Vet_approved Phase 4 302-25-0
11
Benzocaine Approved, Investigational Phase 4 1994-09-7, 94-09-7 2337
12
tannic acid Approved Phase 4 1401-55-4
13
Prednisolone hemisuccinate Experimental Phase 4 2920-86-7
14 Protective Agents Phase 4
15 Gastrointestinal Agents Phase 4
16 Antiemetics Phase 4
17 Neuroprotective Agents Phase 4
18 Anti-Allergic Agents Phase 4
19 Anti-Infective Agents Phase 4
20 Antiviral Agents Phase 4
21 Anti-Bacterial Agents Phase 4
22 Formoterol Fumarate Phase 4
23 Budesonide, Formoterol Fumarate Drug Combination Phase 4
24 Neurotransmitter Agents Phase 4
25 Hormones Phase 4
26 Hormone Antagonists Phase 4
27 glucocorticoids Phase 4
28 Anti-Inflammatory Agents Phase 4
29 Antineoplastic Agents, Hormonal Phase 4
30 Immunoglobulins Phase 4
31 Antibodies Phase 4
32 Antibodies, Monoclonal Phase 4
33 Methylprednisolone Acetate Phase 4
34 Adrenergic beta-Agonists Phase 4
35 Bronchodilator Agents Phase 4
36 Adrenergic Agonists Phase 4
37 Adrenergic Agents Phase 4
38 Tocolytic Agents Phase 4
39 Albuterol Phase 4
40
Albendazole Approved, Vet_approved Phase 3 54965-21-8 2082
41
Lenograstim Approved, Investigational Phase 3 135968-09-1
42
Busulfan Approved, Investigational Phase 3 55-98-1 2478
43
Aldesleukin Approved Phase 3 110942-02-4, 85898-30-2
44
Ketotifen Approved Phase 3 34580-13-7, 34580-14-8 3827
45
Histamine Approved, Investigational Phase 3 51-45-6, 75614-87-8 774
46
Cytarabine Approved, Investigational Phase 3 147-94-4 6253
47
Etoposide Approved Phase 3 33419-42-0 36462
48
Thioguanine Approved Phase 3 154-42-7 2723601
49
Daunorubicin Approved Phase 3 20830-81-3 30323
50
Reslizumab Approved, Investigational Phase 3 241473-69-8

Interventional clinical trials:

(show top 50) (show all 183)
# Name Status NCT ID Phase Drugs
1 Impact of Benralizumab Treatment on Circulating Dendritic Cells in Patients With Eosinophilic Asthma Unknown status NCT03652376 Phase 4 Benralizumab
2 Assessment of Glucocorticoid Responsiveness and Mechanisms of Resistance in Hypereosinophilic Syndromes Completed NCT01524536 Phase 4 prednisone
3 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
4 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
5 Efficacy of Reslizumab Dose Escalation in Patients With Severe Asthma and Persistent Sputum Eosinophilia Despite Standard Dose Therapy Recruiting NCT04710134 Phase 4
6 A Pragmatic Study to Investigate the Efficacy and Safety of Mepolizumab in Severe Uncontrolled Asthma in Brazil Recruiting NCT04228588 Phase 4 Mepolizumab 100 MG [Nucala]
7 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 Recruiting NCT04159519 Phase 4 Symbicort®;Fasenra®;Ventolin®
8 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
9 How to "Choosebetweenamab" for Severe Asthma, Comparing Treatment With Mepolizumab and Omalizumab for Patients With Severe Allergic and Eosinophilic Asthma. Recruiting NCT04585997 Phase 4 Mepolizumab;Omalizumab
10 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) Not yet recruiting NCT04276233 Phase 4 Prednisone/ Prednisolone;Salbutamol
11 Predictive Signature of Benralizumab Response Not yet recruiting NCT04565483 Phase 4 Benralizumab Prefilled Syringe
12 Treatment of Asymptomatic Toxocariasis With Albendazole in Children. A Prospective, Randomized, Placebo-controlled Clinical Trial Unknown status NCT00755560 Phase 3 Albendazole;Placebo
13 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
14 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
15 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
16 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
17 INSPIRE: An Internet-based RCT for Long-term Survivors of Hematopoietic Stem Cell Transplantation Completed NCT00799461 Phase 3
18 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
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 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
21 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
22 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)
23 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
24 Route of Administration of Anti-IL5 Monoclonal Antibody in Prednisone-dependent Eosinophilic Asthma Completed NCT02559791 Phase 2, Phase 3 Placebo
25 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
26 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
27 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
28 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
29 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
30 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
31 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
32 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
33 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
34 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) Recruiting NCT04322604 Phase 3 lirentelimab (AK002)
35 An Open-label Study to Evaluate the Pharmacokinetics and Pharmacodynamics and Long-term Safety of Benralizumab Administered Subcutaneously in Children With Severe Eosinophilic Asthma Recruiting NCT04305405 Phase 3 Benralizumab
36 Benralizumab in Patients With Inadequate Response to Anti-IL5 Monoclonal Antibody Therapies Recruiting NCT03470311 Phase 3
37 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 Recruiting NCT04049175 Phase 3 Treatment A;Treatment B;Treatment C;Treatment D
38 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
39 The Treatment of Down Syndrome Children With Acute Myeloid Leukemia (AML) and Myelodysplastic Syndromes (MDS) Under the Age of 4 Years Active, not recruiting NCT00369317 Phase 3 asparaginase;daunorubicin hydrochloride;cytarabine;thioguanine;etoposide
40 A Double-Blind, Placebo-controlled Clinical Trial to Evaluate Efficacy of Benralizumab in Subjects With Eosinophilic Gastritis Active, not recruiting NCT03473977 Phase 2, Phase 3
41 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
42 A Multinational, Randomized, Double-blind, Parallel-group, Placebo-controlled Study to Investigate the Use of Benralizumab as a Treatment Option for Patients With Bullous Pemphigoid (FJORD) Not yet recruiting NCT04612790 Phase 3
43 An Open Label Extension Study to Evaluate Safety and Efficacy of Mepolizumab in Patients With Hypereosinophilic Syndromes Terminated NCT00097370 Phase 3 mepolizumab
44 Treatment of DRESS (Drug Reaction With Eosinophilia and Systemic Symptoms) With Tegeline® Terminated NCT00505648 Phase 3 Tegeline®
45 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
46 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
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 Open Label Pilot Phase II Study of STI571 in the Treatment of Patients With Idiopathic Hypereosinophilic Syndrome (HES) and Eosinophilic Leukemias Unknown status NCT00276926 Phase 2 STI571
49 An Open-Label, Proof-of-Concept Study to Evaluate the Safety and Efficacy of Dexpramipexole (KNS-760704) in Subjects With Hypereosinophilic Syndrome Unknown status NCT02101138 Phase 2 Dexpramipexole
50 Phase II Study of Imatinib Mesylate in Patients With Life Threatening Malignant Rare Diseases Completed NCT00154388 Phase 2 Imatinib mesylate

Search NIH Clinical Center for Hypereosinophilic Syndrome

Inferred drug relations via UMLS 70 / NDF-RT 51 :


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

Sources

Genetic Tests for Hypereosinophilic Syndrome

About this section

Genetic tests related to Hypereosinophilic Syndrome:

# Genetic test Affiliating Genes
1 Eosinophilia 29
Sources

Anatomical Context for Hypereosinophilic Syndrome

About this section

MalaCards organs/tissues related to Hypereosinophilic Syndrome:

40
Myeloid, Bone Marrow, T Cells, Lung, Neutrophil, Heart, Liver
Sources

Publications for Hypereosinophilic Syndrome

About this section

Articles related to Hypereosinophilic Syndrome:

(show top 50) (show all 21663)
# Title Authors PMID Year
1
Early phase resolution of mucosal eosinophilic inflammation in allergic rhinitis. 61 54
20459697 2010
2
Delayed-type asthmatic response to bronchial challenge with allergen, I: clinical features. 54 61
20486329 2010
3
IL-13 Gene Polymorphisms are Associated With Rhinosinusitis and Eosinophilic Inflammation in Aspirin Intolerant Asthma. 61 54
20358028 2010
4
Airway wall expression of OX40/OX40L and interleukin-4 in asthma. 61 54
20139223 2010
5
Allergen-induced, eotaxin-rich, proangiogenic bone marrow progenitors: a blood-borne cellular envoy for lung eosinophilia. 61 54
20227754 2010
6
Critical role of the platelet-derived growth factor receptor (PDGFR) beta transmembrane domain in the TEL-PDGFRbeta cytosolic oncoprotein. 61 54
20164181 2010
7
Thrombotic storm in Kimura disease. 54 61
19468829 2010
8
Hypereosinophilic syndrome and clonal eosinophilia: point-of-care diagnostic algorithm and treatment update. 61 54
20053713 2010
9
Vernal keratoconjunctivitis--a rare but serious comorbidity of allergic rhinitis and eustachian tube dysfunction. 54 61
19900723 2010
10
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. 54 61
19864487 2010
11
Hyper-IgE syndrome with STAT3 mutation: a case report in Mainland China. 61 54
20490271 2010
12
Concomitant occurrence of kimura disease and mycosis fungoides in a Lebanese woman: significance and response to rituximab. 54 61
19786854 2009
13
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Recent breakthroughs in the understanding and management of chronic eosinophilic leukemia. 61 54
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Changed concepts and definitions of myeloproliferative neoplasms (MPN), myelodysplastic syndromes (MDS) and myelodysplastic/myeloproliferative neoplasms (MDS/MPN) in the updated 2008 WHO classification. 61 54
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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. 61 54
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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. 61 54
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Imatinib has limited therapeutic activity for hypereosinophilic syndrome patients with unknown or negative PDGFRalpha mutation status. 61 54
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A novel FIP1L1-PDGFRA mutant destabilizing the inactive conformation of the kinase domain in chronic eosinophilic leukemia/hypereosinophilic syndrome. 61 54
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Pathogenesis of Churg-Strauss syndrome: recent insights. 54 61
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Eosinophilia-associated muscle disorders: an immunohistological study with tissue localisation of major basic protein in distinct clinicopathological forms. 54 61
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Sources

Variations for Hypereosinophilic Syndrome

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ClinVar genetic disease variations for Hypereosinophilic Syndrome:

6
# Gene Name Type Significance ClinVarId dbSNP ID Position
1 JAK2 , ZBTB20 t(3;9)(q13.31;p24.1) Translocation Likely pathogenic 869413 GRCh37: 3:114069121-114069122
GRCh38:
Sources

Expression for Hypereosinophilic Syndrome

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Search GEO for disease gene expression data for Hypereosinophilic Syndrome.
Sources

Pathways for Hypereosinophilic Syndrome

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

(show all 48)
# Super pathways Score Top Affiliating Genes
1
Innate Immune System 65
Show member pathways
 13.89 RNASE3 PRG2 PDGFRB PDGFRA KIT JAK2
2
ERK Signaling 63
Show member pathways
 13.78 PRG2 PDGFRB PDGFRA KIT JAK2 IL5
3
PEDF Induced Signaling 63
Show member pathways
 13.64 PDGFRB PDGFRA KIT JAK2 IL5 IL3
4
TGF-Beta Pathway 63
Show member pathways
 13.53 PDGFRB PDGFRA KIT JAK2 IL5 IL3
5
Developmental Biology 65
Show member pathways
 13.51 PDGFRB PDGFRA KIT JAK2 IL5 IL3
6
Akt Signaling 63
Show member pathways
 13.34 PDGFRB PDGFRA KIT JAK2 IL5 IL3
7
Response to elevated platelet cytosolic Ca2+ 65
Show member pathways
 13.3 JAK2 IL5 IL3 IL2RA IFNA1 CSF2
8
PAK Pathway 63
Show member pathways
 13.24 PDGFRB PDGFRA KIT JAK2 IL5 IL3
9
Cytokine Signaling in Immune system 65
Show member pathways
 13.18 PDGFRB PDGFRA KIT JAK2 IL5 IL3
10
Phospholipase-C Pathway 63
Show member pathways
 13.08 PRG2 PDGFRB PDGFRA KIT IL3 IL2RA
11
p70S6K Signaling 63
Show member pathways
 13.06 PDGFRB PDGFRA KIT JAK2 IL3 IL2RA
12
PI3K-Akt signaling pathway 36
Show member pathways
 13 PDGFRB PDGFRA KIT JAK2 IL3 IL2RA
13
Ras signaling pathway 36
Show member pathways
 12.72 PDGFRB PDGFRA KIT FGFR1 ABL1
14
Pathways in cancer 36
12.62 PDGFRB PDGFRA KIT JAK2 IL5 IL3
15
NF-kappaB Signaling 4
12.57 KIT JAK2 IL3 IL2RA IFNA1
16
Apoptosis Pathway 69
Show member pathways
 12.46 PDGFRB PDGFRA IL5 IL2RA FGFR1
17
JAK-STAT signaling pathway 36
Show member pathways
 12.4 PDGFRB PDGFRA JAK2 IL5 IL3 IL2RA
18
Phospholipase D signaling pathway 36
Show member pathways
 12.38 PDGFRB PDGFRA KIT CXCL8
19
G-protein signaling RAC1 in cellular process 23
Show member pathways
 12.38 PDGFRB PDGFRA KIT IL3 FGFR1
20
IL-17 Family Signaling Pathways 64
Show member pathways
 12.36 JAK2 IL5 CXCL8 CSF2 CCL11
21
G beta-gamma signalling through PI3Kgamma 65
Show member pathways
 12.19 JAK2 IL5 IL3 IL2RA CSF2
22
Kit receptor signaling pathway 1
Show member pathways
 12.18 KIT JAK2 IL3 CXCL8
23
Tyrosine Kinases / Adaptors 4
12.12 PDGFRB PDGFRA KIT JAK2 FGFR1 ABL1
24
Allograft rejection 36 1
Show member pathways
 12.11 RNASE3 PRG2 PDGFRA IL5 IL3 IL2RA
25
Hematopoietic Stem Cell Differentiation Pathways and Lineage-specific Markers 64
12.03 KIT IL5 IL3 IL2RA CSF2
26
Interleukin-4 and 13 signaling 65
12.01 JAK2 IGHE CXCL8 CCL11
27
Hematopoietic cell lineage 36
11.93 KIT IL5 IL3 IL2RA CSF2
28
Immune response IFN alpha/beta signaling pathway 23
Show member pathways
 11.86 PDGFRB PDGFRA KIT IL5 IL3 IL2RA
29
Cytokine production by Th17 cells in CF (Mouse model) 23
Show member pathways
 11.84 JAK2 CXCL8 CSF2
30
Angiogenesis (CST) 4
11.82 PDGFRB PDGFRA FGFR1
31
Glucocorticoid receptor regulatory network 1
11.79 IL5 CXCL8 CSF2
32
VEGF Pathway (Tocris) 69
Show member pathways
 11.79 PDGFRB KIT FGFR1
33
Calcineurin-regulated NFAT-dependent transcription in lymphocytes 1
Show member pathways
 11.79 IL5 IL3 IL2RA CXCL8 CSF2
34
Innate Lymphoid Cell Differentiation Pathways 64
11.77 KIT IL5 IL2RA
35
Central carbon metabolism in cancer 36
11.77 PDGFRB PDGFRA KIT FGFR1
36
Lung fibrosis 1
11.72 IL5 CXCL8 CSF2 CCL11
37
IL4-mediated signaling events 1
11.71 JAK2 IL5 IGHE CCL11
38
Acute myeloid leukemia 36
11.68 KIT IL3 CSF2
39
Ponatinib Pathway, Pharmacokinetics/Pharmacodynamics 60
Show member pathways
 11.61 PDGFRB PDGFRA KIT ABL1
40
Thymic Stromal LymphoPoietin (TSLP) Signaling Pathway 1
11.56 JAK2 IL2RA CXCL8 CCL11
41
Th2 Differentiation Pathway 64
11.54 JAK2 IL5 IL2RA CCL11
42
Hematopoietic Stem Cell Differentiation 1
11.53 IL5 IL3 CSF2
43
Alzheimers Disease Pathway 63
11.5 PDGFRB PDGFRA FGFR1
44
Vemurafenib Pathway, Pharmacodynamics 60
11.47 PDGFRB PDGFRA FGFR1
45
Pazopanib Pathway, Pharmacokinetics 60
Show member pathways
 11.36 PDGFRB PDGFRA KIT
46
Cytokines and Inflammatory Response 1
11.29 IL5 IL3 CSF2
47
G-protein signaling_Rap2B regulation pathway 23
10.92 PRG2 PDGFRB PDGFRA KIT IL5 IL3
48
Osteoblast Signaling 1
10.85 PDGFRB PDGFRA
Sources

GO Terms for Hypereosinophilic Syndrome

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

# Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 9.61 RNASE3 KIT IL5 IL3 IFNA1 EPX
2 receptor complex GO:0043235 9.46 PDGFRB PDGFRA KIT FGFR1
3 extracellular region GO:0005576 9.36 RNASE3 PRG2 IL5 IL3 IGHE IFNA1

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

(show all 33)
# Name GO ID Score Top Affiliating Genes
1 phosphorylation GO:0016310 10.08 PDGFRB PDGFRA KIT JAK2 FGFR1 ABL1
2 protein phosphorylation GO:0006468 10.04 PDGFRB PDGFRA KIT JAK2 FGFR1 CCL11
3 inflammatory response GO:0006954 10.02 KIT IL5 IL2RA CXCL8 CCL11
4 positive regulation of cell proliferation GO:0008284 10.01 PDGFRB PDGFRA KIT JAK2 IL5 IL3
5 defense response to bacterium GO:0042742 9.96 RNASE3 PRG2 IGHE EPX
6 positive regulation of cell migration GO:0030335 9.95 PDGFRB PDGFRA KIT JAK2 CCL11
7 positive regulation of ERK1 and ERK2 cascade GO:0070374 9.94 PDGFRB PDGFRA CCL11 ABL1
8 positive regulation of protein kinase B signaling GO:0051897 9.92 PDGFRB PDGFRA KIT FGFR1
9 immune response GO:0006955 9.92 PRG2 IL5 IL3 IL2RA IGHE CXCL8
10 transmembrane receptor protein tyrosine kinase signaling pathway GO:0007169 9.88 PDGFRB PDGFRA KIT FGFR1
11 chemotaxis GO:0006935 9.88 RNASE3 PDGFRB PDGFRA CXCL8 CCL11
12 protein autophosphorylation GO:0046777 9.85 PDGFRB PDGFRA KIT JAK2 FGFR1 ABL1
13 positive regulation of peptidyl-tyrosine phosphorylation GO:0050731 9.84 JAK2 IL5 IL3 ABL1
14 cell chemotaxis GO:0060326 9.83 PDGFRB PDGFRA KIT
15 positive regulation of kinase activity GO:0033674 9.83 PDGFRB PDGFRA KIT FGFR1
16 positive regulation of phosphatidylinositol 3-kinase signaling GO:0014068 9.83 PDGFRB PDGFRA KIT JAK2 FGFR1
17 positive regulation of tyrosine phosphorylation of STAT protein GO:0042531 9.81 KIT JAK2 IL3 CSF2
18 positive regulation of MAP kinase activity GO:0043406 9.8 PDGFRB KIT FGFR1
19 phosphatidylinositol-mediated signaling GO:0048015 9.79 PDGFRB PDGFRA FGFR1
20 platelet-derived growth factor receptor signaling pathway GO:0048008 9.76 PDGFRB PDGFRA JAK2 ABL1
21 positive regulation of phosphatidylinositol 3-kinase activity GO:0043552 9.73 PDGFRB PDGFRA KIT
22 peptidyl-tyrosine phosphorylation GO:0018108 9.73 PDGFRB PDGFRA KIT JAK2 FGFR1 ABL1
23 positive regulation of podosome assembly GO:0071803 9.65 IL5 CSF2
24 cardiac myofibril assembly GO:0055003 9.65 PDGFRB PDGFRA
25 retina vasculature development in camera-type eye GO:0061298 9.64 PDGFRB PDGFRA
26 response to fluid shear stress GO:0034405 9.63 PDGFRB CSF2
27 platelet-derived growth factor receptor-beta signaling pathway GO:0035791 9.61 PDGFRB ABL1
28 MAPK cascade GO:0000165 9.61 PDGFRB PDGFRA KIT JAK2 IL5 IL3
29 mast cell chemotaxis GO:0002551 9.59 KIT CCL11
30 metanephric glomerular capillary formation GO:0072277 9.58 PDGFRB PDGFRA
31 positive regulation of cell proliferation by VEGF-activated platelet derived growth factor receptor signaling pathway GO:0038091 9.57 PDGFRB PDGFRA
32 positive regulation of phospholipase C activity GO:0010863 9.46 PDGFRB PDGFRA KIT FGFR1
33 cytokine-mediated signaling pathway GO:0019221 9.32 KIT JAK2 IL5 IL3 IL2RA IGHE

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

# Name GO ID Score Top Affiliating Genes
1 kinase activity GO:0016301 9.88 PDGFRB PDGFRA KIT JAK2 FGFR1 ABL1
2 protein kinase activity GO:0004672 9.73 PDGFRB PDGFRA KIT JAK2 FGFR1 ABL1
3 SH2 domain binding GO:0042169 9.5 KIT JAK2 ABL1
4 transmembrane receptor protein tyrosine kinase activity GO:0004714 9.46 PDGFRB PDGFRA KIT FGFR1
5 platelet-derived growth factor binding GO:0048407 9.43 PDGFRB PDGFRA
6 cytokine activity GO:0005125 9.43 IL5 IL3 IFNA1 CXCL8 CSF2 CCL11
7 vascular endothelial growth factor binding GO:0038085 9.37 PDGFRB PDGFRA
8 protein tyrosine kinase activity GO:0004713 9.1 PDGFRB PDGFRA KIT JAK2 FGFR1 ABL1
Sources

Sources for Hypereosinophilic Syndrome

About this section
3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 20-May-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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