Details of Disease

General Information of Disease (ID: DISVK62S)

Disease Name	Hypereosinophilic syndrome
Synonyms	eosinophilia; hypereosinophilic disorder; hypereosinophilic syndrome; HES; eosinophilic leukocytosis; hypereosinophilic disease
Disease Class	2A20: Myeloproliferative neoplasm
Definition	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 Hierarchy	DIS6SVEE: Syndromic disease DISBOKKP: Eosinophil disorder DIS81H97: Non-familial restrictive cardiomyopathy DISVK62S: Hypereosinophilic syndrome
ICD Code	ICD-11 ICD-11: 2A20.3 ICD-10 ICD-10: D47.5
Disease Identifiers	MONDO ID MONDO_0015691 UMLS CUI C1540912 MedGen ID 280990 Orphanet ID 168956 SNOMED CT ID 1156250009

Drug-Interaction Atlas (DIA) of This Disease

Drug-Interaction Atlas (DIA)

This Disease is Treated as An Indication in 2 Clinical Trial Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
Dexpramipexole	DM6KXAU	Phase 3	Small molecule	[1]
GSK3511294	DMWKSFY	Phase 3	Monoclonal antibody	[2]
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This Disease is Treated as An Indication in 1 Investigative Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
X-081-NAB	DM1TQBN	Investigative	NA	[3]
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Molecular Interaction Atlas (MIA) of This Disease

Molecular Interaction Atlas (MIA)

This Disease Is Related to 2 DTT Molecule(s)

Gene Name	DTT ID	Evidence Level	Mode of Inheritance	REF
JAK1	TT6DM01	Limited	Biomarker	[4]
IL5	TTPREZD	Strong	Biomarker	[5]
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This Disease Is Related to 2 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
RRAGA	OTKISLG4	moderate	Genetic Variation	[6]
RRBP1	OT4ZTPTM	moderate	Biomarker	[6]
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References

1	ClinicalTrials.gov (NCT02101138) An Open-Label, Proof-of-Concept Study to Evaluate the Safety and Efficacy of Dexpramipexole (KNS-760704) in Subjects With Hypereosinophilic Syndrome. U.S.National Institutes of Health.
2	ClinicalTrials.gov (NCT05334368) A Randomized, Double-blind, Placebo-controlled Study to Investigate the Efficacy and Safety of Depemokimab in Adults With Hypereosinophilic Syndrome (HES). U.S.National Institutes of Health.
3	The ChEMBL database in 2017. Nucleic Acids Res. 2017 Jan 4;45(D1):D945-D954.
4	JAK1 gain-of-function causes an autosomal dominant immune dysregulatory and hypereosinophilic syndrome.J Allergy Clin Immunol. 2017 Jun;139(6):2016-2020.e5. doi: 10.1016/j.jaci.2016.12.957. Epub 2017 Jan 19.
5	Mepolizumab-a novel option for the treatment of hypereosinophilic syndrome in childhood.Pediatr Allergy Immunol. 2018 Feb;29(1):28-33. doi: 10.1111/pai.12809. Epub 2017 Nov 28.
6	PDGFRalpha/FIP1L1-positive chronic eosinophilic leukemia presenting with retro-orbital localization: efficacy of imatinib treatment.Cancer Chemother Pharmacol. 2008 Apr;61(4):713-6. doi: 10.1007/s00280-007-0507-7. Epub 2007 Jun 5.