Hypereosinophilic syndrome

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [2]

Synonyms and keywords: HES; Hypereosinophilic disease; Primary hypereosinophilic syndrome; Secondary hypereosinophilic syndrome; Idiopathic hypereosinophilic syndrome


Hypereosinophilic syndrome (HES) is a type of myeloproliferative disorder characterized by a persistently elevated eosinophil count (≥ 1500 eosinophils/mm³) in the blood for at least six months without any recognizable cause, with involvement of either the heart, nervous system, or bone marrow. Hypereosinophilic syndrome is a diagnosis of exclusion, after clonal eosinophilia (such as leukemia) and reactive eosinophilia (in response to infection, autoimmune disease, atopy, hypoadrenalism, tropical eosinophilia, or cancer) have been ruled out. Hypereosinophilic syndrome may be classified into 3 categories: primary, secondary, and idiopathic. Hypereosinophilic syndrome may be caused by either stem cell, myeloid, or eosinophilic neoplasms. Hypereosinophilic syndrome is mainly caused by mutations in the BCR-ABL, PDGFRA, PDGFRβ, and KIT genes. There are some associations with chronic eosinophilic leukemia as it shows similar characteristics and genetic defects. Patients with hypereosinophilic syndrome may be initially asymptomatic. Early clinical features include fatigue, diarrhea, and rash. Prognosis is generally poor, and the 3-year survival rate of patients with hypereosinophilic syndrome is approximately 12%. Findings related with poor prognosis in hypereosinophilic syndrome, include: presence of anemia, thrombocytopenia, white blood cell count greater than 100,000 cells/cm3, abnormal marrow and/or basophils, elevated serum levels of vitamin B12, serum tryptase, and abnormal levels of leukocyte alkaline phosphatase. If left untreated, hypereosinophilic syndrome is progressively fatal. Treatment will depend on the presence of mutations. Treatment of choice for patients with FIP1L1/PDGFRA mutation is imatinib and first line treatment for patients without FIP1L1/PDGFRA mutation is glucocorticoid therapy. The addition of the monoclonal antibody mepolizumab may reduce the dose of glucocorticoids. Once diagnosed and successfully treated, patients with hypereosinophilic syndrome are followed-up periodically every 6 or 12 months depending on the clinical progression. Follow-up testing includes the following tests: complete blood count, biochemical profile (liver enzymes, creatine kinase, renal function, and troponin), electrocardiogram, and tissue biopsies.

Historical Perspective

  • Hypereosinophilic syndrome was first described by Hardy and Anderson in 1968.[1]
  • In 2003, PDGFRA and FIP1L1 gene mutations were first identified in the pathogenesis of hypereosinophilic syndrome.[2]


  • Hypereosinophilic syndrome may be classified into 3 categories:.[3]
  • Primary hypereosinophilic syndrome
  • Also known as neoplasic hypereosinophilic syndrome
  • Secondary hypereosinophilic syndrome
  • Also known as reactive hypereosinophilic syndrome
  • Idiopathic hypereosinophilic syndrome


  • PDGFRA gene
  • PDGFRB gene
  • FGFR1 gene


Microscopic Pathology
Images shown below are courtesy of Professor Peter Anderson DVM PhD and published with permission. © PEIR, University of Alabama at Birmingham, Department of Pathology

Gross Pathology
Images shown below are courtesy of Professor Peter Anderson DVM PhD and published with permission. © PEIR, University of Alabama at Birmingham, Department of Pathology


  • Hypereosinophilic syndrome may be caused by either stem cell, myeloid, or eosinophilic neoplasms.
  • Hypereosinophilic syndrome is caused by a mutation in the BCR-ABL, PDGFRA, PDGFRβ, and KIT gene.[3]

Differentiating Hypereosinophilic Syndrome from Other Diseases

Epidemiology and Demographics

  • Hypereosinophilic syndrome is a very rare disease.
  • The prevalence of hypereosinophilic syndrome is approximately 0.36 to 6.3 per 100,000 individuals worldwide.


  • Patients of all age groups may develop hypereosinophilic syndrome.
  • Hypereosinophilic syndrome is more commonly observed among adults.



  • Caucasian individuals are more likely to develop cancer of unknown primary origin.[6]

Risk Factors

Natural History, Complications and Prognosis


Diagnostic Criteria

  • The diagnosis of hypereosinophilic syndrome includes the following findings:[3]


  • Symptoms of hypereosinophilic syndrome may include the following:[3]

Physical Examination

Laboratory Findings

Imaging Findings

  • There are no imaging findings associated with hypereosinophilic syndrome.

Other Diagnostic Studies


Medical Therapy




  1. Hardy WR, Anderson RE (1968). "The hypereosinophilic syndromes". Ann. Intern. Med. 68 (6): 1220–9. PMID 5653621.
  2. Cools J, DeAngelo DJ, Gotlib J, et al. (2003). "A tyrosine kinase created by fusion of the PDGFRA and FIP1L1 genes as a therapeutic target of imatinib in idiopathic hypereosinophilic syndrome". N. Engl. J. Med. 348 (13): 1201–14. doi:10.1056/NEJMoa025217. PMID 12660384.
  3. 3.0 3.1 3.2 3.3 3.4 3.5 3.6 Gleich GJ, Leiferman KM (2009). "The hypereosinophilic syndromes: current concepts and treatments". Br. J. Haematol. 145 (3): 271–85. doi:10.1111/j.1365-2141.2009.07599.x. PMID 19243381.
  4. 4.0 4.1 4.2 4.3 Weller PF (1991). "The immunobiology of eosinophils". N. Engl. J. Med. 324 (16): 1110–8. doi:10.1056/NEJM199104183241607. PMID 2008184.
  5. Chusid MJ, Dale DC, West BC, Wolff SM (1975). "The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature". Medicine (Baltimore). 54 (1): 1–27. doi:10.1097/00005792-197501000-00001. PMID 1090795.
  6. 6.0 6.1 6.2 6.3 6.4 Hypereosinophilic syndrome: an update. http://www.pneumonologia.gr/articlefiles/20070228_Hypereosinophilic_Syndrome_An_Update.pdf Accessed on April 4, 2016