Hypereosinophilic syndrome (ICD 10 - D72.1) is a syndrome in which the main diagnostic criterion is an increase in the number of blood cells belonging to the leukocyte group, detected in the bloodstream and causing, in turn, organ dysfunction. At the moment, in the scientific community, pathology is not considered as an independent nosological unit. Nevertheless, the diagnosis, symptoms and treatment of hypereosinophilic syndrome are of interest to many.
Epidemiology
Despite the fact that this syndrome is most found in adults, children are no exception and, according to statistics, among them boys get sick much more often than girls in a ratio of 4:1.
For a detailed analysis of the topic, it is necessary to recall the main functions of eosinophils:
- Eosinophilic granulocytes are among the effector cells of inflammation in the body, according to recent clinical studies.
- The granulocytes released by the granulocyte maintain the microbicidal potentialinfluence on both foreign substances and surrounding tissues.
- Eosinophils play a key role in allergic reactions and in building anthelmintic immunity.
- Participate in maintaining tissue and immunological homeostasis.
Hypereosinophilic syndrome in childhood is often caused by an allergic trigger, but can also be caused by autoimmune processes, hemato- and oncopathology. In the development of this pathology, a genetic genesis is also distinguished - in children, this problem may be associated with trisomy of the 8th or 21st chromosome.
Classification of hypereosinophilic syndrome
By etiological factor:
- Reactive eosinophilia.
- Idiopathic hypereosinophilic syndrome.
For the detection of immunoglobulins in the blood:
- Immunoglobulin-dependent eosinophilia is caused by specific IgE.
- Immunoglobulin-independent.
By predominance in a particular disease:
- Myeloproliferative.
- Lymphoproliferative.
Myeloproliferative variant presents with the following symptoms in patients:
- elevated vitamin B12;
- myelofibrosis;
- spelenomegaly;
- response to imatinib (tyrosine kinase inhibitor);
- anemia;
- thrombocytopenia.
Lymphoproliferative variant is caused by clonal rearrangement of T-cell receptor genes and is characterized by the following symptoms:
- CEC (circulating immunecomplexes);
- hypergammaglobulinemia (IgE);
- response to corticosteroid group treatment;
- angioedema;
- Skin anomalies.
What are the normal levels of eosinophils in the blood?
The normal content of eosinophilic granulocytes in peripheral blood ranges from 1 to 5 percent of the total number of leukocytes. In absolute terms, this value is from 120 to 350 cells in 1 microliter.
Newborn babies may have physiological eosinophilia of more than 700 cells per 1 µl, which is also considered a normal blood eosinophil count.
What will be the main criteria for setting pathology?
First, it is extremely important to understand that eosinophilia is established by obtaining the absolute values of eosinophilic granulocytes in peripheral blood, and according to this number, three degrees of eosinophilia are distinguished:
- I degree: slight eosinophilia (from 500 to 1500 cells in 1 microliter).
- II degree: moderate eosinophilia (from 1500 to 5000 cells in 1 microliter).
- III degree: severe eosinophilia (more than 5000 cells in 1 microliter).
Peripheral blood eosinophilia >1500/microliter that lasts 6 months or more (!) is the main criterion for diagnosis.
Clinic
Non-specific symptoms include malaise, anorexia, weight loss, abdominal pain, myalgia, fever, weakness inbody, that is, asthenization occurs.
To identify the etiological factor, it is necessary to establish the leading clinical syndrome, based on the main symptoms of the disease:
- The hematological syndrome is the leading one and is characterized by: anemia, lymphadenopathy, splenomegaly, thrombocytopenia, thromboemboli.
- Intoxication syndrome will be expressed in diseases such as: myeloproliferative pathologies, lymphogranulomatosis, lymphocytic leukemia.
- Bronchopulmonary (bronchial asthma, periarthritis nodosa, bronchopulmonary aspergillosis).
- Cardiopulmonary syndrome is characterized by the formation of parietal thrombi with emboli.
- Gastrointestinal syndrome is characterized by symptoms such as abdominal cramps, loose stools, and vomiting.
- Skin syndrome may present with atopic dermatitis, angioedema, pruritus, urticaria, dermatographism.
The damage to organs in this syndrome is due to their infiltration by eosinophils, which can lead to multiorgan dysfunction. Organs such as the heart, skin, spleen, nervous system, and lungs may be involved.
Pathogenesis
Experts identify the main mechanisms. This is:
- Antibody-dependent chemotaxis that develops during helminthic invasions (this is evidenced by the appearance of IgE and IgG).
- Tumor processes, some of which may release eosinophilic chemotactic factor.
- Tumor eosinophilia (leukemia).
Howrecognize?
Diagnosis of hypereosinophilic syndrome is based on the exclusion of other possible causes of eosinophilia. For example, infectious, parasitic. That is, it is a diagnosis of exclusion and is made if the etiology of this phenomenon could not be established.
The main laboratory and instrumental methods to diagnose this syndrome are as follows:
- Leukogram indicating the absolute number of eosinophilic granulocytes.
- Blood biochemistry (liver enzymes, creatine kinase, GFR, urea, troponin, acute phase proteins).
- Immunology of hypereosinophilic syndrome. Indicators such as antinuclear antibodies, cationic proteins, IgE, lymphogram.
- Stool analysis for cysts, eggs.
- Electrocardiography.
- Echocardiography.
- Instrumental examination of the respiratory organs (radiography).
- Computed tomography of the chest and abdomen.
- In an examination such as a bone marrow puncture, both mature eosinophils and progenitor cells will be detected.
- Also performs a neurological examination, which includes: examination of the child, checking reflexes, electroencephalography, examination of the fundus.
Forecast
Unfavorable prognosis for hypereosinophilic syndrome in children in most cases is due to complications that are usually manifested by dysfunction of certain organs - most often, this is the heart. Heart failure can lead to disability and even death.
Treatment of pathology
Treatmentbegins with the appointment of the glucocorticosteroid prednisolone, followed by imatinib, drugs that regulate the content of eosinophils, for example, Interferon-alpha and Etoposide.
"Imatinib" is an antitumor agent, an inhibitor of tyrosine kinase, an enzyme. Synthesized in chronic myeloid leukemia.
"Etoposide" is an anticancer drug that has a cytotoxic effect. It must be remembered that this medicine has restrictions for use: it is contraindicated for children under two years of age due to the fact that in clinical studies its safety for children under two years of age and, in principle, its effectiveness has not been proven).
The effect of glucocorticoids is to inhibit the growth of proliferation of the eosinophilic germ of granulocytes, their activation factors. Leukotriene inhibitors, phosphodiesterase inhibitors, myelosuppressive drugs can also be used for this purpose.
Supportive therapy is used for symptoms that indicate that the heart is also involved in the process - this can manifest itself as infiltrative cardiomyopathy, valvular heart disease, heart failure). Anticoagulants, antiplatelet drugs ("Aspirin", "Clopidogrel") can be used.
Required consultation of specialists in order to choose the right treatment. Seek help from the followingdoctors: hematologist (selects intensive care for the patient), dermatologist (his treatment tactics are necessary for skin manifestations of the syndrome), neurologist (involved in the process when neurological disorders appear), cardiologist, pulmonologist.
Conclusion
It must be remembered that hypereosinophilic syndrome requires qualified medical care. In no case should such a pathology be ignored, since in case of complications it often leads to death.
It is not worth hoping that everything will go away on its own - only timely access to a doctor and proper treatment can guarantee the success of therapeutic measures.
