A Name and info...

I finally heard my dr use a name I could look up and identify. Here's some info about my disorder more specifically...

Hypereosinophilic Syndrome (HES) is a group of disorders where very high numbers of eosinophils are found in peripheral blood counts (blood test) and organ tissue damage occurs. Unlike eosinophilic gastrointestinal disorders (EGID), which only affects the digestive tract, HES may affect any organ in the body. To diagnose HES, more than 1500 eosinophils/microliters must be found in the blood for more than six months with injury (damage) to organs. Chronic eosinophilic leukemia (CEL) means the eosinophils are clonal (all from the same cell line, identical).
Treatment of Hypereosinophilic Syndromes
Treatment of hypereosinophilic syndromes (HES) and chronic eosinophilic leukemia (CEL).
In the hypereosinophilic syndromes, high numbers of eosinophils are found in the blood and affect multiple organs in the body. This may include the stomach and intestines, the heart, lungs, skin and others. The eosinophils cause inflammation and eventually damage to the involved organs.

Treatment will vary based on type of disease, organs involved and disease severity.
1. Glucocorticoids (“Steroids”) Higher dose systemic (oral) steroids are often needed to control HES with organ involvement. Steroids are medications that fight (suppress) many types of inflammation. They are not specific for suppressing eosinophils, although eosinophils are particularly sensitive to them. Steroids can be taken intravenously (IV), or ingested orally. Systemic steroids, those that are absorbed into the bloodstream (oral or IV), are very effective for treating a number of eosinophilic disorders. Unfortunately, the disease may return when the steroids are stopped. Steroids given in this manner may have many harmful side effects when used for long periods of time. Serious side effects can include osteoporosis (brittle bones from bone loss), infections, adrenal insufficiency (body becomes unable to properly respond to illness or stress), avascular necrosis (collapse of the bones in a joint, usually the hip), and stunted growth. Common side effects may include fluid retention (swelling), increased appetite, “moon-face”, and irritability.

2. GleevecTM (Imatinib Mesylate) was developed to treat certain types of leukemia. Imatinib may induce remission in select types of HES. Not all patients with HES will respond to Imatinib. Genetic testing (for FIP1L1-PDGFRα gene rearrangement) can help determine if Gleevec is likely to help.
3. Calcineurin Inhibitors The calcineurin inhibitors include cyclosporine (Neoral®, Sandimmune®, Gengraf®) and tacrolimus (Prograf®). These are very potent medications that suppress the immune system by interfering with the function of T cells. They are used primarily to prevent organ rejection in people who have had organ transplants. They may also be of benefit in some patients with the hypereosinophilic syndrome. Because calcineurin inhibitors have a number of potentially harmful side effects, they are reserved for more severe and refractory (treatment-resistant) cases. Side effects include kidney failure, nerve damage, headaches, hair loss or excess growth, elevated cholesterol, high blood pressure, diabetes and development of cancer. Blood levels of these medications must be carefully monitored. Many other medications, particularly antibiotics, can affect the blood levels.

4. Anti-Neoplastic Agents Agents used to treat cancers are not specific for eosinophilic disorders, but may be helpful in some types of HES. These are potent medications with potentially harmful side effects and are reserved for more severe disease. Careful monitoring while taking these medications is very important.

Chemotherapeutic agents and approaches that have been used in HES include:
Methotrexate
Hydroxyurea
Cyclophosphamide
etoposide
Vincristine
Bone marrow transplant

Details of these potent medications are beyond the scope of this review. Further information can be found at www.cancereducation.com.


Tests to diagnose HES include a complete blood count and examination of other organs based on symptoms or other abnormal test results. Additional tests usually include a complete blood count and eosinophil count, blood samples for liver and kidney function, a blood test for Vitamin B12, erythrocyte sedimentation rate (general sign of inflammation), and a blood test for tryptase. Depending on symptoms and test results, other studies may be performed. For instance, ultrasound (echocardiography) is used to look at the function of the heart. A chest x-ray may be done to examine the lungs. As always, your medical team can best guide the necessary diagnostic tests on an individual basis.


Idiopathic Hypereosinophilic Syndrome
Criteria for diagnosis include:
1. Peripheral blood eosinophilia (high numbers of eosinophils in the blood) more than 1500 eosinophils/, for at least six months’ duration.
2. End-organ (heart, lungs, GI tract, brain, skin, etc) involvement with eosinophil tissue infiltration (invasion) and injury.
3. No other known causes for the eosinophilia (e.g. parasitic infections).

What are the prospects for people with HES?
Prospects for people with HES vary from person to person. It depends on how severe the disease is, which bodily organs are affected, and the success of treatment. In some cases, HES may be fatal.
 
Can HES be eliminated from the body?
HES cannot be eliminated, but it can often be treated.
 
How is HES treated?
Treatment of HES depends on which bodily organs are involved and how severe the disease is. The most common treatment for HES consists of high doses of steroids (medications in the cortisone family). Other treatments include medications that suppress the immune system, and anticancer therapies.