Hypereosinophilic Syndrome

Hypereosinophilic syndrome (HES) is a type of blood disorder. It happens when your body has too many eosinophils. Eosinophils are a type of white blood cell that's part of your immune system. When there are too many, these cells can travel to different parts of your body. Over time, this extra buildup of eosinophils can damage the organs they reach.

Commonly affected organs include the skin, lungs, gut (digestive tract), heart, blood vessels, and nervous system. If left untreated, hypereosinophilic syndrome can become a serious and even life-threatening condition.

Early signs of a condition called HES might include feeling very tired, having a cough, trouble breathing, aches in your muscles, a skin rash, and a fever.

Some cases of hypereosinophilic syndrome are inherited, meaning they tend to appear in multiple family members. Other cases are linked to specific types of cancers, infections, or other health issues. Essentially, there are different reasons why someone might develop this condition. For some, it seems to be passed down through generations, while for others, there's an underlying health problem that triggers it.

Hereditary Encephalopathy with Striatal and Neuronal Changes (HES) can happen to anyone, but it's more common in men, typically diagnosed between the ages of 20 and 50. This means that while women can be affected, men in this age range are at a slightly higher risk. It's important to remember that this is a general observation, and individual cases can vary.

High eosinophil counts can happen for many reasons, including infections, allergies, and reactions to medicines. If a doctor suspects hypereosinophilic syndrome (HES), they'll likely ask about your travel history and any medications you're taking. This helps them figure out if something else is causing the high eosinophil count.

To get a better understanding of what's going on, your doctor might order some tests. These could include:

• Blood tests: These can help find out if you have an autoimmune disease, a parasitic infection, or problems with your liver or kidneys. Autoimmune diseases happen when your body mistakenly attacks its own tissues. Parasitic infections are caused by tiny organisms that live in or on your body.

• Allergy tests: These tests can pinpoint environmental or food allergies that might be contributing to the high eosinophil count. Knowing what you're allergic to can help with treatment and avoiding triggers.

• Stool tests: These check for parasitic infections, like hookworm. Hookworms are a type of parasite that can live in the intestines.

• Genetic tests: In some cases, a genetic test can look for a specific gene mutation that's linked to HES. This helps doctors understand the underlying cause of the disorder.

• Imaging tests: These tests create pictures of your body's internal structures. Some examples include:

  • X-rays: These are useful for checking the health of your lungs.
  • CT scans: These create detailed images of your chest, abdomen, and pelvis, helping doctors look for problems in these areas.
  • Echocardiograms or MRIs: These specialized imaging tests help doctors assess the health of your heart.

Mayo Clinic has a team of experts who can provide comprehensive care for people with HES. They can help you understand your condition and develop a personalized treatment plan.

Hypereosinophilic syndrome (HES) treatment focuses on lowering the number of eosinophils in your blood to stop damage, particularly to your heart. The best approach depends on several factors: how severe your symptoms are, how serious the condition is, and what might be causing the syndrome.

If you have mild symptoms and your eosinophil count is already low, you might only need regular checkups to watch for any HES-related changes. No medication might be necessary.

Medications:

• Corticosteroids (like prednisone): These are often the first medications doctors try. Corticosteroids are powerful anti-inflammatory drugs. They help reduce inflammation throughout the body, which can help manage HES.
• Other options: If corticosteroids don't work, or if your symptoms are severe, other medicines might be used, such as:
  • Hydroxyurea (Droxia, Hydrea, Siklos): This medicine can help stop the body from making too many eosinophils.
  • Imatinib (Gleevec): This drug targets certain proteins that can cause the issue.
  • Vincristine: This is a chemotherapy drug that can be used to control the syndrome in some cases.

Blood Thinners: Because HES can increase your risk of blood clots, you might need blood-thinning medications like warfarin (Coumadin) to prevent these clots.

More Advanced Treatments:

• Stem Cell or Bone Marrow Transplant: In situations where other treatments haven't worked, a stem cell or bone marrow transplant may be considered. This is a major procedure that replaces your blood-forming cells with healthy ones. This is a last resort option, used only when other treatments haven't been successful.

It's crucial to discuss your specific situation with your doctor to determine the best treatment plan for you. The severity, symptoms, and cause of your HES will all be considered.