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idiopathic hypereosinophilic syndrome - Clinical Profile, Treatments & Trials | Kisho | Kisho

Features include very common findings: Increased total eosinophil count; and common findings: Pallor, Low red blood cell count (anemia), Fever, and Elevated white blood cell count (increased total leukocyte count) and others. 83 total HPO annotations.

Organ System	Phenotype Count	Example Features
Digestive system	14	Enlarged liver (hepatomegaly), Enlarged spleen (splenomegaly), Abdominal distention
Brain and nerves	13	Nervous system problems (abnormality of the nervous system), Myeloproliferative disorder, Atypical behavior
Heart and blood vessels	10	Restrictive cardiomyopathy, Endocardial fibrosis, Myocardial eosinophilic infiltration
Lungs and breathing	7	Pulmonary infiltrates, Respiratory distress, Dyspnea
Skin	7	Pruritus, Eczematoid dermatitis, Urticaria
Blood and immune system	7	Enlarged spleen (splenomegaly), Low red blood cell count (anemia), Elevated white blood cell count (increased total leukocyte count)
Bones and joints	5	Joint inflammation (arthritis), Joint swelling, Arthralgia
Muscles	3	Myalgia, Muscle weakness, Skeletal muscle atrophy
Metabolism	1	Fever
Eyes	1	Blurred vision
Growth and development	1	Failure to thrive
Lab test results	1	Elevated circulating hepatic transaminase concentration

Treatment

Compiled from FDA-approved drug records, orphan drug designations, and FDA Adverse Event Reporting System (FAERS) safety data. GeneReviews management guidance is included where available.

1 FDA-approved treatment is available for idiopathic hypereosinophilic syndrome, including IMATINIB MESYLATE (GLEEVEC, approved 2003).

Approved Therapies

Brand Name	Generic Name	Mechanism	Approved	Market Status
GLEEVEC	IMATINIB MESYLATE	—	2003	Available

Gene therapy approaches for idiopathic hypereosinophilic syndrome have been reported in the published literature.

Clinical Trials

1 trial found

Explore Trials

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

1 clinical trial registered. Interventions under study include drug therapy. Pipeline includes 1 PHASE2. Research is primarily sponsored by academic and government institutions.

59 publications have been identified in PubMed for idiopathic hypereosinophilic syndrome. Research spans Case Report / Case Series (68%), Review / Meta-Analysis (12%), and Diagnostic / Biomarker (7%).

Research Type	Count	% of Total
Patient case studies	40	68%
Research summaries	7	12%
Testing and diagnosis research	4	7%
Other research	3	5%
Laboratory research	2	3%
Disease patterns and progression	2	3%
New treatment approaches	1	2%

Recent Publications

M V (2026). [PMID: 41728149](https://pubmed.ncbi.nlm.nih.gov/41728149/). *Indian J Hematol Blood Transfus*. [Other]
Parimoo A (2026). [PMID: 42113356](https://pubmed.ncbi.nlm.nih.gov/42113356/). *Int J Cardiovasc Imaging*. [Case Report / Case Series]
Jha SK (2026). [PMID: 32491456](https://pubmed.ncbi.nlm.nih.gov/32491456/). *Unknown Journal*. [Case Report / Case Series]
AlShamrani AA (2026). [PMID: 41994071](https://pubmed.ncbi.nlm.nih.gov/41994071/). *J Cardiol Cases*. [Case Report / Case Series]
Nemes A (2026). [PMID: 41898261](https://pubmed.ncbi.nlm.nih.gov/41898261/). *Biomedicines*. [Basic Science / Preclinical]
Zhang B (2026). [PMID: 41307472](https://pubmed.ncbi.nlm.nih.gov/41307472/). *Clin Nucl Med*. [Diagnostic / Biomarker]
Zhang J (2026). [PMID: 41945721](https://pubmed.ncbi.nlm.nih.gov/41945721/). *Clin Lab*. [Case Report / Case Series]
Jain P (2026). [PMID: 41488420](https://pubmed.ncbi.nlm.nih.gov/41488420/). *J Allergy Clin Immunol Glob*. [Epidemiology / Natural History]
Kurup A (2026). [PMID: 41784094](https://pubmed.ncbi.nlm.nih.gov/41784094/). *Ann Indian Acad Neurol*. [Case Report / Case Series]
Rossi V (2026). [PMID: 41700233](https://pubmed.ncbi.nlm.nih.gov/41700233/). *Cureus*. [Case Report / Case Series]

Kisho

idiopathic hypereosinophilic syndrome

Overview

Clinical Presentation

Medical References

External Resources

OMIM

Orphanet

GARD

Questions for Your Care Team

Questions for Your Care Team

Explore clinical features

Genetic Landscape

Diagnosis

Treatment

Approved Therapies

Clinical Trials

Prognosis

Research Landscape

Recent Publications

Latest News & Developments

Sustained low disease activity during long-term mepolizumab treatment in FIP1L1::PDGFRA-negative hypereosinophilic syndrome: a multicenter real-world study.

Incidence and prevalence of hypereosinophilic syndrome in the United States: A retrospective claims database study.

Ruxolitinib for life-threatening corticosteroid-refractory hypereosinophilic syndrome: A multicenter case series.

idiopathic hypereosinophilic syndrome

Overview

Clinical Presentation

Medical References

External Resources

OMIM

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

Explore clinical features

Genetic Landscape

Diagnosis

Treatment

Approved Therapies

Clinical Trials

Prognosis

Research Landscape

Recent Publications

Latest News & Developments

Sustained low disease activity during long-term mepolizumab treatment in FIP1L1::PDGFRA-negative hypereosinophilic syndrome: a multicenter real-world study.

Incidence and prevalence of hypereosinophilic syndrome in the United States: A retrospective claims database study.

Ruxolitinib for life-threatening corticosteroid-refractory hypereosinophilic syndrome: A multicenter case series.