At the Hypereosinophilic syndrome there are several multisystem diseases that are relatively rare. They are characterized by the fact that eosinophilia occurs in the peripheral blood for a period of more than six months. Alternatively, bone marrow oosinophilia is also possible, the cause of which cannot be proven. In addition, there is a severe dysfunction of the organs in connection with eosinophilic tissue infiltration. The hypereosinophilia syndrome is also known in many cases by the abbreviation HES. Synonyms for the disease are, for example, eosinophilic reticulosis or eosinophilic leukemoid.
What is Hypereosinophilia Syndrome?
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According to Definion that is Hypereosinophilic syndrome characterized in that the eosinophilic granulocytes, which are located in the peripheral blood, multiply over more than six months. The increase in granulocytes extends to the bone marrow. This results in a dysfunction of the organs.
Basically, hypereosinophilia syndrome is a very rare disease, the causes of which are still largely unexplained according to the current state of medical research. The damage to the organs results primarily from long-term eosinophilia of the blood and bone marrow.
Hypereosinophilia syndrome has an estimated frequency of approximately one to nine 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 occurs 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 researched. 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 the hypereosinophilia 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 in molecular biology and can only be determined in this way.
The damage to the organs that occurs in the context of the hypereosinophilia syndrome results primarily 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 in the context of hypereosinophilia syndrome cover a wide range. They vary considerably depending on the patient, the extent 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. With these, for example, restrictive changes to the lungs are possible. In the majority of cases, the hypereosinophilia syndrome results in symptoms that are related to the heart, skin and 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 nodes, pruritus, papulovesicles or angioedema. Erythroderma develops rarely.
In individual cases, digital necrosis and the so-called Raynaud's syndrome were also observed in the context of the hypereosinophilia syndrome. If the heart is involved in the disease, eosinophilic endo- and myocarditis usually occurs. In principle, impairment of the heart is the most common cause of death in hypereosinophilia syndrome.
Endomyocardial necrosis and, later, thrombotic changes occur. Endomyocardial fibrosis is possible at a later stage. In addition, the hypereosinophilia syndrome leads to coughing, lung infiltrates and pleural effusions. In many cases, the intellectual capacity of the affected person decreases.
Diagnosis & course of disease
In order to diagnose hypereosinophilic syndrome, a thorough medical history of the affected patient is required. The attending physician discusses, among other things, possible previous illnesses and the symptoms of the respective patient. This is followed by physical examinations and laboratory tests, for example of blood and urine.
In addition, a bone marrow puncture may be recommended to detect the multiplication of eosinophils. Echocardiography provides information about a possible involvement of the heart in the disease. The attending physician may also consider performing a myocardial biopsy. In the context of the differential diagnosis, parasitoses on the skin, Churg-Strauss syndrome and eosinophilic leukemia, for example, must be excluded.
Complications
Hypereosinophilia syndrome usually causes a number of different complaints and complications. In some cases, however, those affected do not suffer from symptoms, which makes diagnosis and thus early treatment of this disease very difficult. The affected person usually loses weight and has a severe 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 itching can be affected. The person concerned feels weak and exhausted and therefore no longer actively takes part in social life.
It is not uncommon for a strong cough to develop, which limits the performance and resilience of the patient. 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 of the symptoms can be eliminated without any consequential damage, so that life expectancy is not affected by hypereosinophilia syndrome.
When should you go to the doctor?
A doctor's visit is necessary as soon as breathing disorders develop. If you experience breathlessness, interruptions in breathing or breathing difficult, you should consult a doctor. If the feeling of lack of oxygen occurs, if there is 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. A racing heart, tightness in the chest or increased blood pressure are warning signs from the organism that need to be clarified. If the skin turns slightly bluish, if there is internal tension for a long time or if irritability sets in, a doctor must be consulted.
Loss of appetite and severe weight loss are cause for concern. If food is refused for several days or weeks, there is a risk of the body being 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, a check-up visit with a doctor is advisable. If an overexertion situation occurs unusually quickly or if the person concerned experiences a general malaise, a doctor should be consulted. In the event of a diffuse feeling of illness or a loss of cognitive performance, a doctor should clarify the symptoms.
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Treatment & Therapy
The therapeutic measures are based on the severity and severity of the hypereosinophilia syndrome. In some cases, the active ingredient imatinib is used, otherwise various corticosteroids are available. PUVA therapy also appears to be an effective treatment method. Oral anticoagulation is recommended to prevent embolism. In principle, external therapy for hypereosinophilia syndrome is symptomatic.
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➔ Medicines for painOutlook & forecast
The prognosis for hypereosinophilia syndrome varies and is often very difficult to assess. In many patients the disease proceeds without symptoms. Whether they will suffer long-term organ damage and which organs are affected depends on many factors that are not yet fully understood. In about 50 percent of cases, the disease is severe from the start, and life-threatening complications can occur. The skin, nervous system, lungs, or heart are often affected. These organs can be seriously damaged by the increased occurrence of thrombi, fibrosis and organ infarction.
In severe cases, life expectancy can only be increased through intensive therapy, which reduces the number of eosinophilic granulocytes in the blood. As with leukemia, the treatment is chemotherapy. Cortisone and other drugs are also given. At the same time, the damaged organs must also be treated. In extreme cases, it can be fatal, especially if the heart is affected.
In very severe cases, a bone marrow transplant may be necessary. Life expectancy can be increased through these intensive treatment measures. However, the quality of life is greatly reduced. In addition to the severe health restrictions caused by organ involvement, there are also unpleasant side effects of chemotherapy. Unfortunately, those affected cannot lead a normal life at the moment. However, new active ingredients with fewer side effects are being worked on.
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 consult a suitable specialist immediately at the first characteristic signs and symptoms of the disease so that adequate treatment can be initiated quickly.
Aftercare
In the case of hypereosinophilia syndrome, there are no direct measures and options for follow-up care available to those affected. As a rule, an early diagnosis is necessary in order to relieve the symptoms correctly and permanently. Since hypereosinophilia syndrome is a hereditary disease, genetic counseling should be carried out if you want to have children in order to prevent the disease from being passed on.
In most cases, medication is used to treat the disease. The person concerned should pay attention to a correct and regular intake and also pay attention to possible interactions. With children, parents should always check the correct intake. If you have any questions or are unclear, a doctor should always be contacted or consulted first.
Most of the time, support and help from friends and family is also necessary. This can also prevent psychological upsets or depression. If there are severe symptoms, 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. A complete healing can often not be achieved.
You can do that yourself
With hypereosinophilia syndrome, there are unfortunately no special ways to help yourself. Direct medical treatment is also not possible in many cases, so that only the individual complaints can be restricted.
In many cases the patients suffer from severe loss of appetite. However, regular intake of food and fluids should also be ensured in order to avoid dehydration and various deficiency symptoms. Should deficiency symptoms occur anyway, they can be combated with the help of supplements. Since hypereosinophilia syndrome can also lead to heart problems, the heart should not be unnecessarily stressed. Therefore, strenuous sports or activities should be avoided in order not to unnecessarily burden the circulation. Furthermore, patients should take part in regular examinations by various doctors in order to avoid complications. Discomfort on the skin 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 common home remedies.
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