Learn About Hypereosinophilic Syndrome

What is the definition of Hypereosinophilic Syndrome?
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.
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What are the alternative names for Hypereosinophilic Syndrome?
  • HES
  • Hypereosinophilic syndrome, idiopathic
What are the causes of Hypereosinophilic Syndrome?
When the term hypereosinophilic syndrome (HES) was originally coined in 1975, the condition was thought to be 'idiopathic' or of an unknown cause. Today, in approximately 3/4 of cases, the underlying cause still remains unknown. However, recent advances in diagnostic techniques have lead researchers to believe that some people affected by HES may have eosinophilia due to a variety of causes, including:
  • Myeloproliferative neoplasms or other disorders that affect the bone marrow (myeloproliferative disorders). This form is called myeloproliferative HES.
  • Increased production of interleukin-5 (a protein produced by certain types of white blood cell). This form is called lymphocytic HES.
  • A change (mutation) in an unknown gene passed down through a family. This form is called familial HES.
What are the symptoms of Hypereosinophilic Syndrome?
The signs and symptoms of hypereosinophilic syndrome can vary significantly depending on which part(s) of the body are affected. Frequent symptoms listed by body system include:
  • Skin - rashes, itching, and edema.
  • Lung - asthma, cough, difficulty breathing, recurrent upper respiratory infections, and pleural effusion.
  • Gastrointestinal - abdominal pain, vomiting, and diarrhea.
  • Musculoskeletal - arthritis, muscle inflammation, muscle aches, and joint pain.
  • Nervous system - vertigo, paresthesia, speech impairment, and visual disturbances.
  • Heart - congestive heart failure, cardiomyopathy, pericardial effusion, and myocarditis.
  • Blood - deep venous thrombosis, and anemia.
Affected people can also experience a variety of non-specific symptoms such as fever, weight loss, night sweats and fatigue.
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What are the current treatments for Hypereosinophilic Syndrome?
The treatment and management of hypereosinophilic syndrome (HES) varies based on the severity of the condition and if an underlying cause for the high levels of white blood cells (eosinophilia) has been identified.  Medications have been developed that target specific causes of HES. For HES without an identified cause, treatment is focused on managing the symptoms. Specialists involved in the care of someone with HES may include:
  • Hematologist
  • Oncologist
  • Immunologist
  • Infectious disease specialist
  • Cardiologist
  • Pulmonologist
Who are the top Hypereosinophilic Syndrome Local Doctors?
Elite
Highly rated in
10
conditions

University Of Lille

Lille, FR 

Guillaume Lefevre is in Lille, France. Lefevre is rated as an Elite expert by MediFind in the treatment of Hypereosinophilic Syndrome. He is also highly rated in 10 other conditions, according to our data. His top areas of expertise are Hypereosinophilic Syndrome, Eosinophilic Granulomatosis with Polyangiitis, Vasculitis, and Aromatase Deficiency.

Elite
Highly rated in
17
conditions
Infectious Disease

National Institutes Of Health

Bethesda, MD 

Amy Klion is an Infectious Disease doctor in Bethesda, Maryland. Dr. Klion is rated as an Elite doctor by MediFind in the treatment of Hypereosinophilic Syndrome. She is also highly rated in 17 other conditions, according to our data. Her top areas of expertise are Hypereosinophilic Syndrome, Loiasis, Spirurida Infections, and Secernentea Infections. She is licensed to treat patients in Maryland.

 
 
 
 
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Elite
Highly rated in
19
conditions

Hôpital Foch

Suresnes, FR 

Matthieu Groh is in Suresnes, France. Groh is rated as an Elite expert by MediFind in the treatment of Hypereosinophilic Syndrome. He is also highly rated in 19 other conditions, according to our data. His top areas of expertise are Hypereosinophilic Syndrome, Eosinophilic Granulomatosis with Polyangiitis, Granulomatosis with Polyangiitis, and Vasculitis.

How is Hypereosinophilic Syndrome diagnosed?

A diagnosis of hypereosinophilic syndrome is based on the following criteria:

  • Persistent eosinophilia, as defined by blood counts showing increased numbers of eosinophils (greater than 1500 eosinophils/uL) for at least 6 months
  • Signs and symptoms of organ involvement
  • No evidence of other conditions that can cause eosinophilia*
*Due to advances in the diagnostic techniques, a specific cause of eosinophilia can be identified in a proportion of cases that would have otherwise been classified as idiopathic hypereosinophilic syndrome
Is Hypereosinophilic Syndrome an inherited disorder?
Although most cases of hypereosinophilic syndrome (HES) are not inherited, some cases do appear to be passed down through a family. In these families, the exact underlying genetic cause is unknown, but the genetic change (mutation) is thought to be inherited in an autosomal dominant manner. In autosomal dominant conditions, an affected person only needs a change (mutation) in one copy of the responsible gene in each cell. In some cases, an affected person inherits the mutation from an affected parent. Other cases may result from new (de novo) mutations in the gene. These cases occur in people with no history of the disorder in their family. A person with an autosomal dominant condition has a 50% chance with each pregnancy of passing along the altered gene to his or her child.
What are the latest Hypereosinophilic Syndrome Clinical Trials?
A Multicentre Single Arm Observational Prospective Study to Assess Demographic Characteristics Burden of Disease PRO's in Uncontrolled, Severe Eosinophilic Asthma Patients Aged 18y or Older and Qualifying for Treatment With Benralizumab in BE
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Efficacy of Mepolizumab in Patients With Late-onset Severe Eosinophilic Asthma and Fixed Obstruction
What are the Latest Advances for Hypereosinophilic Syndrome?
IL13 May Play an Important Role in Developing Eosinophilic Chronic Rhinosinusitis and Eosinophilic Otitis Media with Severe Asthma.
Anticoagulant Use as an Independent Risk Factor and Higher In-Hospital Mortality in Patients Showing Alveolar Hemorrhage in Diffuse Lung Disease.
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Mepolizumab in Hypereosinophilic Syndrome: A Systematic Review and Meta-analysis.