Hypereosinophilic Syndrome : Causes-Symptoms-Diagnosis-Treatment


 What Is Hypereosinophilic Syndrome (HES)?

Hypereosinophilic syndrome (HES) refers to a rare group of conditions that are associated with chronic eosinophilia with proof of organ involvement. Signs and symptoms vary notably based totally on which components of the frame are affected. Although any organ machine can be worried in HES, the coronary heart, significant nervous machine, skin, and respiration tract are the most usually affected. The circumstance was firstly started to be "idiopathic" or of unknown cause. However, current advances in diagnostic testing have allowed a purpose to be recognized in about 1 / 4 of cases.

Hypereosinophilic syndrome (HES) is a rare circumstance caused by an overload of white blood cells referred to as eosinophils. Eosinophils guard your frame from parasitic infections and reply to allergens. Normally, eosinophils account for five% to 7% of your white blood cells, or about 100 to 500 eosinophils in step with microliter of blood. When you have certain situations, inclusive of hypersensitive reactions, your eosinophils multiply to cause a circumstance known as eosinophilia.

What Is Hypereosinophilic Syndrome (HES)?
Hypereosinophilic Syndrome 

Hypereosinophilic syndrome occurs whilst eosinophilia accelerates, speeding up eosinophil manufacturing and growing the quantity of eosinophils. This eosinophil overload can damage your organs, which include your coronary heart, lungs, pores and skin and nervous gadget. Left untreated, hypereosinophilic syndrome may be existence-threatening. Fortunately, prompt analysis and treatment mean greater than 80% of those identified with hypereosinophilic syndrome are alive 5 years after analysis.

  1. Circulatory system

  1. Heart

  2. Arteries

  3. Veins

  4. Blood vessels

Medical terms

  • Hypereosinophilic (hy-in keeping with-ee-o-SIN-o-fill-ik) syndrome (HES) is a group of blood issues that occur when you have excessive numbers of eosinophils — white blood cells that play an critical position for your immune device. Over time, the excess eosinophils input various tissues, in the end damaging your organs.
  • The most not unusual objectives are the skin, lungs, digestive tract, coronary heart, blood and apprehensive machine. Untreated, HES can end up life-threatening.
  • The hypereosinophilic syndrome (HES) is characterized by the presence of marked unexplained blood and tissue eosinophilia related to an expansion of scientific manifestations. Since 1975, three standards had been used to define HES: (1) blood eosinophilia ≥1500/mm3 for longer than 6 months (or demise before 6 months related to signs and symptoms and signs of hypereosinophilic ailment), (2) loss of evidence for parasitic, allergic, or different recognized causes of eosinophilia, and (three) presumptive signs and symptoms of organ involvement, which includes coronary heart failure, gastrointestinal dysfunction, relevant nervous gadget abnormalities, fever, or weight reduction.

Hypereosinophilic syndrome (HES) is a disorder in which the bone marrow makes too many eosinophils a type of white blood cell Eosinophils are found in all body tissues and normally help fight infections by engulfing and destroying foreign substances and parasites In HES they are usually present in blood bone marrow other organs and the skin This disorder occurs most often in men between the ages of 30 and 60 years old.

(HES) Hypereosinophilic syndrome (HES) also known as hypereosinophilic disorder (HED) is a rare and serious condition that causes eosinophil blood cells to accumulate in the blood Eosinophils are white blood cells that fight infection Symptoms of Hypereosinophilic Syndrome .

Is hypereosinophilic syndrome serious?

Hypereosinophilic syndrome is not a serious condition In fact the prognosis for hypereosinophilic syndrome is very good with proper treatment Patients who have been diagnosed with hypereosinophilic syndrome may feel anxious about their condition but they can take comfort in knowing that this disease is treatable when it's identified and treated early Treatment options include corticosteroids and immunosuppressive agents.

What is acute hypereosinophilic syndrome?

Acute hypereosinophilic syndrome (AHE) is a rare life-threatening disorder characterized by the overproduction of specific cells that fight parasitic infections In AHE these cells known as eosinophils accumulate in the blood and other tissues of the body while white blood cells attempt to destroy them As a result people with AHE may develop severe inflammation and damage to the heart lungs liver bones and skin.

Symptoms Hypereosinophilic syndrome (HES)

The maximum not unusual HE'S symptom is a rash. Hypereosinophilic signs correlate (match to) to the location of your frame stricken by high eosinophil stages. For instance, if your coronary heart has a surprisingly high degree of eosinophils, you might have signs of congestive coronary heart failure, cardiomyopathy, myocarditis and pericardial effusion. Likewise, if the eosinophils are congregating on your lungs, you would possibly have routine (repeating) top respiration infections, coughing and issue breathing.

Early signs of HIS might also consist of fatigue, cough, breathlessness, muscle pain, rash and fever.

These symptoms include:

  • Skin rashes such as urticaria or angioedema

  • Dizziness

  • Memory loss or confusion

  • Cough

  • Shortness of breath

  • Fatigue

  • Fever

  • Mouth sores

Causes Hypereosinophilic syndrome (HES)

Some sorts of hypereosinophilic syndrome have a tendency to run in families. Other types were related to certain varieties of cancers, infections or different fitness issues.Researchers don’t know all the factors that cause the dramatic growth in eosinophils that causes maximum instances of hypereosinophilic syndrome. They have, although, identified some conditions or occasions that is probably accountable:

  • Myeloproliferative diseases: These diseases are characterized by excessive blood cellular manufacturing to your bone marrow.

  • High levels of interleukin-5: This is a protein produced by your white blood cells.

  • A genetic abnormality that drives cell growth.

Risk factors Hypereosinophilic syndrome (HES)

Hypereosinophilic syndrome is a disorder in which excess levels of eosinophils a type of white blood cell are found in the bloodstream Normally eosinophils are rare in healthy people but they make up 5 to 10 percent of circulating blood cells In hypereosinophilic syndrome however eosinophils may account for as much as 30 to 40 percent of the total number of white blood cells Eosinophils are a key part of the immune system because they fight infection by ingesting certain bacteria and viruses.

HES can have an effect on anyone. But it happens more frequently in guys, commonly among the long time of 20 and 50.

Diagnosis Hypereosinophilic syndrome (HES)

Healthcare companies use a technique of elimination to diagnose HES. For example, if you have a chronic itchy rash, your healthcare provider could in all likelihood do checks to check for positive pores and skin issues and prescribe remedies for those issues.

If you’re still having symptoms, your healthcare provider might test your blood for high eosinophil ranges. For instance, in case your signs imply troubles along with your liver, and your healthcare issuer has ruled out other causes, they may carry out a liver feature blood test. They may also carry out a biopsy to affirm a hypereosinophilic syndrome prognosis.

Many kinds of disorders can improve your eosinophil stage, along with positive infections, allergic reactions and reactions to medications. When trying to decide whether you have got hypereosinophilic syndrome (HES), your medical doctor is likely to invite approximately your journey history and any medicines you're taking, to assist rule out these different causes.

Laboratory tests

Your physician may additionally need records from some of the subsequent lab exams:

  • Blood tests, To hit upon autoimmune situations, parasitic infections, or troubles along with your liver or kidneys

  • Allergy tests, to detect environmental or food allergies

  • Stool tests, to detect parasitic infections such as hookworm

  • Genetic test, to check for a gene mutation that can cause HES

Imaging tests

Imaging tests may include:

  • X-rays, to check the condition of your lungs

  • CT scan, to detect problems in the chest, abdomen and pelvis

  • Echocardiogram or MRI, to assess heart function

Treatment Hypereosinophilic syndrome (HES)

The aim of HIS treatment is to lessen eosinophil tiers within the blood and tissues, thereby preventing tissue damage–mainly inside the heart. Standard HES remedy includes glucocorticosteroid medications inclusive of prednisone, and chemotherapeutic agents including hydroxyurea, chlorambucil and vincristine. Interferon-alpha can also be used as a treatment. This medicine ought to be administered by using common injections.

Research is uncovering new therapies for HES. One new approach for controlling malignant cell increase is the usage of tyrosine kinase inhibitors including Gleevec (imatinib). Monoclonal antibody therapy, such as alemtuzumab (anti-CD52) has additionally proven promise for treatment of HES. In reality, based totally on a latest phase III randomized, placebo-controlled trial1, the monoclonal antibody Nucala or mepolizumab (anti-IL-five) has just obtained approval by the U.S. Food and Drug Administration for remedy of adults and youngsters aged 12 years and older with HES for six months or longer without every other identifiable non-blood related purpose of the ailment.

Treatment for hypereosinophilic syndrome is geared toward decreasing your eosinophil count to save you tissue damage, especially to your heart. Specific remedy depends on your signs, the severity of your situation and the reason for your HES.

If you don't have any symptoms and your eosinophil matter is low sufficient, you would possibly require no remedy aside from near monitoring for any changes related to HES.


Systemic corticosteroids, which include prednisone, are the primary line treatment. Other treatment alternatives include:

  • Hydroxyurea (Droxia, Hydrea, Siklos)

  • Imatinib (Gleevec)

  • Vincristine

Because HES can increase your hazard of blood clots, you could possibly additionally be prescribed blood-thinning medicines such as warfarin (Coumadin).

Surgery and other procedures

If nothing else has labored, your medical doctor may endorse a stem mobile or bone marrow transplant.

  1. Cardiac rehabilitation and circulatory rehabilitation

Preparing for your appointment

You'll likely first bring your symptoms to the attention of your circle of relatives. Depending on your signs, you will be noted as a professional in blood illnesses (hematology), coronary heart situations (cardiology) or allergic reactions.

Consider taking a relative or friend alongside to the appointment to help take into account all the statistics provided.

Here's some records that will help you get geared up for the appointment, and what to anticipate from the doctor.

What you can do

Before your appointment, make a list of:

  • Signs and symptoms, Which include any that appear unrelated to the purpose for the appointment

  • Any medications, Consisting of vitamins, herbs and over-the-counter drug treatments that you're taking, and their dosages

  • Key personal information, including any major stresses or recent changes in your life

For hypereosinophilic syndrome, some primary questions to ask the doctor encompass:

  • Will I need additional tests?

  • What are the treatment options?

  • What are the benefits and risks of each treatment?

  • Should I see additional specialists? What will that cost, and will my insurance cover it?

  • Are there brochures or other printed material that I can have? What websites do you recommend?

What to expect from your doctor

The physician is in all likelihood to invite you numerous questions. Be ready to answer them to permit time later to cover different factors you need to deal with. Examples include:

  • When did you first begin experiencing symptoms?

  • Are the symptoms continuous or occasional?

  • How severe are the symptoms?

  • What, if anything, seems to improve your symptoms?

  • What, if anything, appears to worsen the symptoms?

  • Have you traveled out of the country lately?

  • Do you have any allergies or skin conditions?

  • Have you been exposed to any parasites such as hookworm?

General summary

  1. There is no cure for hypereosinophilic syndrome Treatment focuses on managing signs and symptoms.
  2. Eosinophils are a type of white blood cell that protects the body from infections However they can also cause inflammation in people with allergies or asthma Antihistamines and corticosteroids reduce the amount of eosinophils in the body.
  3. Hypereosinophilic syndrome (HES) is a rare condition in which an abnormal increase in certain white blood cells called eosinophils causes them to accumulate in the bone marrow blood and body tissues The excess of these white blood cells causes inflammation and tissue damage HES is a type of chronic eosinophilia which is characterized by the presence of high concentrations of eosinophils in the blood for more than six months The specific cause of hypereosinophilic syndrome remains unknown However researchers believe that genetic predisposition or immune system dysfunction may play roles in this disorder There is no cure for HES but treatments can help reduce symptoms and prevent complications.

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