Hypereosinophilic Syndrome Resource - Frequently Asked Questions About Hypereosinophilic Syndrome

What causes an increase in eosinophils?

What is Idiopathic Hypereosinophilic Syndrome (HES)?

How many people are currently living with HES?

How do I know if I have HES? What are the signs and symptoms?

What treatments are available for HES?

What type of doctor treats the symptoms of HES?

What is an eosinophil?

Eosinophils, one of five types of white blood cells, are part of the body's defense system against parasites and are also involved in allergic responses.

What causes an increase in eosinophils?

High levels of eosinophils can occur within the body for a number of reasons. These can include allergic reactions, parasitic infections, or other disruptions to the body's immune response.

What is idiopathic Hypereosinophilic Syndrome (HES)?

HES is a rare group of disorders characterized by persistent and marked buildup of eosinophils in the blood and organs. People with HES have a large number of white blood cells for no apparent reason. HES can affect any number of organs including the heart, lungs, intestines, and skin. People with HES may have a broad range of symptoms including weakness, fatigue, cough, rash, or may experience rapid onset of life-threatening cardiac symptoms, which can often make the condition difficult to accurately diagnose.

How many people are currently living with HES?

Current estimates suggest that it affects between 2,000 and 5,000 individuals in the U.S. Challenges in diagnosing the condition mean there may be more individuals living with the condition than estimated. Since the symptoms of HES are similar to other diseases, people with HES may be experiencing symptoms and unknowingly living with the condition for some time before they are properly diagnosed. Diagnosis generally occurs between the ages of 20 and 50. HES is also more common in men than women.

How do I know if I have HES? What are the signs and symptoms?

People with HES can have a wide range of general symptoms, including weakness, fatigue, cough, shortness of breath, muscle pain, rash, fever, diarrhea, or cardiac symptoms. The symptoms can be similar to those of other diseases and include a wide range of persistent nonspecific symptoms, similar to those of a cold or the flu, which can eventually lead to organ damage in the heart, central nervous system, blood, lungs, skin, and gastrointestinal tract. A doctor may need to rule out these other conditions before he or she can accurately diagnose you as having HES. As a result, it can take months to years before a person is officially diagnosed with HES.

How is HES diagnosed?

The main criteria for diagnosing HES include:

Ruling out an allergic reaction or an infection from a parasite
Extremely high blood eosinophilia levels (exceeding 1,500 cells/uL) for 6 months or more
Organ problems and damage as a result of too many eosinophils

If you have any symptoms, especially those that cause you to alter your daily activities, you should seek advice from your doctor.

Is there a cure for HES?

There is no cure for HES. The primary objective for treatment of HES is long-term disease control aimed at reducing eosinophil levels to prevent organ damage and life-threatening blood clots.

What treatments are available for HES?

There is no cure for HES. The primary objective for treatment of HES is long-term disease control aimed at reducing eosinophil levels to prevent organ damage and life-threatening blood clots.

Treatment options:

Systemic corticosteroids are commonly prescribed therapy, but long-term use is limited by potentially serious side-effects.
Tyrosine Kinase Inhibitors are a type of medication that is used primarily to treat cancers, but have also been found to be effective in some patients with HES.
Second-line options include cytotoxic and immunomodulatory agents. These agents have limitations in terms of safety and tolerability.

While these therapies cannot cure HES, they can help reduce the symptoms of the disease. There are also investigational therapies currently being studied for the treatment of HES.

What type of doctor treats the symptoms of HES?

Patients with HES may see one or more of the following doctors, depending on how their condition affects them:

Allergist - Specializes in the prevention, diagnosis and treatment of immune system problems such as allergies, asthma, inherited immunodeficiency diseases, autoimmune diseases and even HIV/AIDS. Another name for an allergist is an immunologist.
Cardiologist - Specializes in the diagnosis and treatment of diseases or conditions of the heart and blood vessels.
Dermatologist - Evaluates and manages conditions that affect the skin, hair and nails.
Gastroenterologist - Specializes in the diagnosis and treatment of conditions affecting the digestive system, including the colon and intestines.
Hematologist - Specializes in the diagnosis and treatment of diseases of the blood and the blood system.
Immunologist - See Allergist.
Pulmonologist - Treats problems with the lungs and airways, such as difficulty breathing or chronic cough.
Rheumatologist - Specializes in the diagnosis and treatment of arthritis and other diseases of the joints, muscles and bones.