HES Definition and Meaning


According to abbreviationfinder, HES stands for Hypereosinophilia Syndrome. Hypereosinophilic syndrome is a multisystem disease that is relatively rare. They are characterized by the presence of eosinophilia in the peripheral blood for more than six months. Alternatively, bone marrow osinophilia is also possible, the cause of which cannot be proven. In addition, there is a strong dysfunction of the organs in connection with eosinophilic tissue infiltrates. The hypereosinophilia syndrome is often referred to with the abbreviation HES. Synonyms for the disease are, for example, eosinophilic reticulosis or eosinophilic leukemoid.

What is Hypereosinophilia Syndrome?

A doctor’s visit is necessary as soon as breathing disorders appear. If you experience shortness of breath, pauses in breathing or difficult breathing, you should see a doctor. If the feeling of lack of oxygen sets in, if there is a fear of suffocation or if the person concerned begins to hyperventilate, a doctor should be consulted.

According to Definion, hypereosinophilia syndrome is characterized by an increase in eosinophilic granulocytes, which are located in the peripheral blood, for more than six months. The proliferation of granulocytes extends to the bone marrow. From this follows a dysfunction of the organs.

Basically, the hypereosinophilia syndrome is a very rare disease, the causes of which are still largely unclear according to the current state of medical research. The damage to the organs results primarily from a long-lasting eosinophilia of the blood and the bone marrow.

Hypereosinophilic syndrome occurs with an estimated frequency of approximately 1 to 9 cases in 100,000 people. Men are nine times more likely to be affected by the disease than women. In the majority of cases, the disease appears between the ages of 20 and 50. In principle, a distinction is made between different types of hypereosinophilia syndrome, for example the idiopathic, the familial, the lymphocytic or the myeloproliferative HES.

Causes

The potential causes of hypereosinophilia syndrome have not yet been fully explored. In some cases, the affected patients show mutations in the so-called PDGFRA gene. A fusion gene is formed, which disrupts the maturation of myeloid cells. In addition, there is another theory about possible causes for the development of hypereosinophilic syndrome. A special interleukin is increasingly produced, which leads to the expansion of a population of so-called T-lymphocytes. The potential causes have been researched using molecular biology and can only be determined in this way.

The damage to the organs that occurs as part of the hypereosinophilic syndrome primarily results from various toxic substances that are associated with the eosinophilic granulocytes. These substances lead to fibrosis, thrombi and infarcts of the organs.

Symptoms, Ailments & Signs

The typical symptoms and complaints that can occur as part of the hypereosinophilia syndrome show a wide range. They vary considerably depending on the patient, the type and severity of the disease. On the one hand, there are people in whom the disease does not show any symptoms.

On the other hand, some patients experience severe to life-threatening symptoms of hypereosinophilia syndrome. In these, for example, restrictive changes to the lungs are possible. In the majority of cases, the hypereosinophilia syndrome causes complaints that are related to the heart, the skin and the nervous system.

In addition, the disease often shows general symptoms such as fever, weight loss and loss of appetite. The possible symptoms on the skin are varied. The affected patients sometimes suffer from reddened nodules, pruritus, papulovesicles or angioedema. Rarely does erythroderma develop.

In individual cases, digital necrosis and what is known as Raynaud’s syndrome have also been observed as part of the hypereosinophilia syndrome. If the heart is involved in the disease, eosinophilic endo- and myocarditis usually results. In principle, the impairment of the heart is the most common cause of death in hypereosinophilia syndrome.

This leads to endomyocardial necrosis and, as the disease progresses, to thrombotic changes. At a later stage, endomyocardial fibrosis is possible. Coughing, pulmonary infiltrates and pleural effusions also occur as part of the hypereosinophilia syndrome. In many cases, the intellectual capacity of those affected decreases.

Diagnosis & course of disease

With regard to the diagnosis of hypereosinophilia syndrome, a thorough medical history of the affected patient is first required. The attending doctor discusses, among other things, possible previous illnesses and the symptoms of the respective patient. This is followed by physical examinations and laboratory tests, such as blood and urine.

In addition, a bone marrow biopsy may be recommended to demonstrate the proliferation of eosinophils. An echocardiography provides information about a possible involvement of the heart in the disease. In addition, the treating physician may consider performing a myocardial biopsy. As part of the differential diagnosis, for example, parasitosis on the skin, Churg-Strauss syndrome and eosinophilic leukemia must be ruled out.

Complications

Hypereosinophilic syndrome usually leads to a number of different symptoms and complications. In some cases, however, those affected do not suffer from any symptoms, which makes the diagnosis and thus the early treatment of this disease very difficult. The affected person usually loses weight and suffers from a strong fever.

In addition, there is a loss of appetite, from which malnutrition can continue to develop. This can have a negative effect on the general condition and on the internal organs. The skin is reddened and may be itchy. The affected person feels weak and exhausted and therefore no longer actively participates in social life.

It is not uncommon for a strong cough to develop, which limits the patient’s performance and resilience. Ordinary activities and sporting activities are also no longer possible without further ado. Treatment of hypereosinophilia syndrome takes place with the help of drugs.

There are no further complaints or complications. If treatment is started early, most symptoms can be resolved without sequelae, so life expectancy is not affected by hypereosinophilic syndrome.

When should you go to the doctor?

A doctor’s visit is necessary as soon as breathing disorders appear. If you experience shortness of breath, pauses in breathing or difficult breathing, you should see a doctor. If the feeling of lack of oxygen sets in, if there is a fear of suffocation or if the person concerned begins to hyperventilate, a doctor should be consulted. As soon as breathing becomes worse, a medical evaluation should be initiated immediately. Heart palpitations, tightness in the chest or increased blood pressure are warning signs of the organism that need to be clarified. If the skin turns slightly bluish, if there is internal tension over a longer period of time or if irritability sets in, a doctor must be consulted.

Loss of appetite, as well as a sharp loss of weight, is a cause for concern. If food intake is denied for several days or weeks, there is a risk that the body will be undersupplied. A doctor’s visit is necessary so that the cause of the symptoms can be found and a life-threatening condition can be ruled out. If physical or sporting activities can no longer be carried out as usual, it is advisable to see a doctor for a check-up. If an overexertion situation sets in unusually quickly or if the person concerned experiences a general malaise, a doctor should be consulted. If there is a diffuse feeling of illness or a loss of cognitive performance, a doctor should clarify the symptoms.

Treatment & Therapy

The therapeutic measures depend on the degree of severity and the extent of the hypereosinophilic syndrome. In some cases, the active ingredient imatinib is used, otherwise various corticosteroids are available. PUVA therapy also seems to be an effective treatment method. Oral anticoagulation is recommended to prevent embolism. In general, external therapy for hypereosinophilia syndrome is symptomatic.

Outlook & Forecast

The prognosis of hypereosinophilia syndrome varies and is often very difficult to assess. In many patients, the disease is asymptomatic. Whether they suffer long-term organ damage and which organs are then affected depends on many factors that have not yet been fully elucidated. In about 50 percent of cases, the disease is difficult from the start, and life-threatening complications can occur. The skin, nervous system, lungs or heart are often affected. These organs can be severely damaged by an increased occurrence of thrombi, fibrosis and organ infarctions.

In severe cases, life expectancy can only be increased by intensive therapy that reduces the number of eosinophilic granulocytes in the blood. The treatment is like leukemia with chemotherapy. In addition, cortisone and other drugs are administered. At the same time, the damaged organs must also be treated. Especially if the heart is affected, it can lead to death in extreme cases.

In very severe cases, a bone marrow transplant may be necessary. Life expectancy can be increased by these intensive treatment measures. However, the quality of life is greatly reduced. In addition to the severe health restrictions caused by the organ involvement, there are also unpleasant side effects of chemotherapy. Unfortunately, those affected are not yet able to lead a normal life. However, work is being done on new active ingredients with fewer side effects.

Prevention

Effective measures to prevent hypereosinophilia syndrome are not known according to the current state of medical research. It is all the more important to see a suitable specialist as soon as the first characteristic signs and symptoms of the disease appear, so that appropriate treatment can be initiated quickly.

Aftercare

In the case of hypereosinophilia syndrome, there are no direct measures or options for aftercare available to those affected. As a rule, an early diagnosis is necessary in order to alleviate the symptoms properly and permanently. Since hypereosinophilia syndrome is an inherited disease, genetic counseling should be carried out if you wish to have children in order to prevent the disease from being passed on.

In most cases, the disease is treated by taking medication. The person concerned should pay attention to correct and regular intake and also pay attention to possible interactions. In the case of children, parents should always check that they are taking it correctly. If you have any questions or are unclear, always contact or consult a doctor first.

In most cases, the support and help of friends and family is also necessary. This can also prevent mental upsets or depression. If severe symptoms occur, a doctor must be consulted immediately. In the worst case, the affected person can die prematurely, so that the life expectancy of the person affected is also reduced by this disease. Complete healing can often not be achieved.

You can do that yourself

Unfortunately, there are no special options for self-help with hypereosinophilia syndrome. Direct medical treatment is also not possible in many cases, so that only the individual complaints can be restricted.

In many cases, patients suffer from severe loss of appetite. However, regular intake of food and liquids should also be ensured in order to prevent dehydrationand to avoid various deficiencies. Should deficiency symptoms nevertheless occur, they can be combated with the help of supplements. Since hypereosinophilia syndrome can also lead to heart problems, the heart should not be put under unnecessary strain. Therefore, strenuous sports or activities should be avoided in order not to unnecessarily burden the circulatory system. Furthermore, the patients should take part in regular examinations by different doctors in order to avoid complications. Skin problems can be avoided and treated by using creams and ointments.

Since it can often lead to sore throats, extreme temperatures should also be avoided. Cough or hoarseness can be treated with the usual home remedies.

Hypereosinophilia Syndrome

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