Hyper-eosinophilic 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 group of leukocytes, detected in the bloodstream and, in turn, causing 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 often found in adults, children are no exception and, according to statistics, among them, boys get sick much more often than girls in a 4: 1 ratio.
For a detailed discussion of the topic, it is necessary to recall the main functions of eosinophils:
- According to recent clinical studies, scientists include eosinophilic granulocyte among the effector cells of inflammation in the body.
- Granules released by granulocytes maintain a microbicidal potential that affects both foreign substances and surrounding tissues.
- Eosinophils play a key role in allergic reactions and in the creation of anthelmintic immunity.
- Participate in the maintenance of tissue and immunological homeostasis.
The hypereosinophilic syndrome in childhood is often caused by an allergic trigger, but can also be caused by autoimmune processes, hemato-and oncopathology. Genetic origin is also distinguished in the development of this pathology - in children this problem can be associated with trisomy of the 8th or 21st chromosome.
Classification of hypereosinophilic syndrome
By etiological factor:
- Reactive eosinophilia.
- Idiopathic hypereosinophilic syndrome.
By detecting immunoglobulins in the blood:
- Immunoglobulin-dependent eosinophilia causes specific IgE.
- Immunoglobulin-independent.
By prevalence in a particular disease:
- Myeloproliferative.
- Lymphoproliferative.
The myeloproliferative variant is manifested by the following symptoms in patients:
- elevated levels of vitamin B12;
- myelofibrosis;
- spelenomegaly;
- reaction to treatment with imatinib (tyrosine kinase inhibitor);
- anemia;
- thrombocytopenia.
The lymphoproliferative variant is due to a clonal rearrangement of T cell receptor genes and is characterized by the following symptoms:
- CEC (circulating immune complexes);
- hypergammaglobulinemia (IgE);
- reaction to treatment with a group of corticosteroid drugs;
- angioedema;
- abnormalities from the skin.
What are the normal levels of eosinophils in the blood?
The normal content of eosinophilic granulocytes in the peripheral blood ranges from 1 to 5 percent of the total number of leukocytes. In absolute terms, this value is equal to from 120 to 350 cells in 1 microliter.
In newborns, physiological eosinophilia may be observed, amounting to more than 700 cells per 1 ฮผl, which is also considered the normal content of eosinophils in the blood.
What will relate to the main criteria for the formulation of pathology?
Firstly, it is extremely important to understand that eosinophilia is established when absolute values โโof eosinophilic granulocytes are obtained in peripheral blood and three degrees of eosinophilia are distinguished by this number:
- 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, which lasts 6 months or more (!) - The main criterion for diagnosis.
Clinic
Non-specific symptoms include malaise, anorexia, weight loss, abdominal pain, myalgia, fever, weakness in the body, that is, asthenia occurs.
To identify the etiological factor, it is necessary to establish a leading clinical syndrome based on the main symptoms of the disease:
- Hematological syndrome is leading and is characteristic of it: anemia, lymphadenopathy, splenomegaly, thrombocytopenia, thromboembolism.
- Intoxication syndrome will be expressed in diseases such as: myeloproliferative pathologies, lymphogranulomatosis, lymphocytic leukemia.
- Bronchopulmonary (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 colic in the abdomen, loose stools, and vomiting.
- Skin syndrome can manifest itself as atopic dermatitis, angioedema, pruritus, urticaria, and dermatographism.
Organ damage in this syndrome is due to their eosinophil infiltration, which can lead to multi-organ dysfunction. Organs such as the heart, skin, spleen, nervous system, and lungs may be involved.
Pathogenesis
Experts identify the main mechanisms. It:
- Antibody-dependent chemotaxis developing during helminthic invasions (this is indicated by the appearance of IgE and IgG).
- Tumor processes, in some of which an eosinophilic chemotactic factor can be released.
- Tumor eosinophilia (leukemia).
How to recognize
Diagnosis of hypereosinophilic syndrome is based on the exclusion of other possible causes of eosinophilia. For example, infectious, parasitic. That is, this is the 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, proteins of the acute phase of inflammation).
- Immunology of hypereosinophilic syndrome. Indicators such as antinuclear antibodies, cationic proteins, IgE, lymphogram.
- Analysis of feces for the presence of cysts, eggs.
- Electrocardiography
- Echocardiography.
- Instrumental examination of the respiratory system (radiography).
- Computed tomography of the chest and abdominal cavities.
- In a study such as bone marrow puncture, both mature eosinophils and progenitor cells will be detected).
- He also conducts a neurological examination, which includes: examination of the child, reflex check, electroencephalography, fundus examination.
Forecast
An unfavorable prognosis for hypereosinophilic syndrome in children in most cases is caused by complications, which are usually manifested by dysfunction of various organs - most often, this is the heart. Damage to the heart can lead to disability and even death.
Pathology treatment
Treatment begins with the administration of prednisone glucorticosteroid, followed by the use of imatinib, drugs that regulate the content of eosinophils, for example, Interferon-alpha and Etoposide.
"Imatinib" - an antitumor agent, an inhibitor of tyrosine kinase, an enzyme. It is synthesized in chronic myeloid leukemia.
"Etoposide" is an antitumor drug that has a cytotoxic effect. It must be remembered that this medicine has limitations for use: for children under two years of age it is contraindicated due to the fact that in clinical trials its safety for children under two years of age and, in principle, have not been proven effective).
The effect of glucocorticoids is to inhibit the growth of proliferation of the eosinophilic sprout of granulocytes, their activation factors. For these purposes, leukotriene inhibitors, phosphodiesterase inhibitors, myelosuppressive drugs can also be used.
Supportive therapy is used for symptoms that indicate that the heart is also involved in the process - this can manifest itself in the form of infiltrative cardiomyopathy, heart valve damage, heart failure). Anticoagulants, antiplatelet drugs (Aspirin, Clopidogrel) can be used.
Consultation of specialists is necessary in order to choose the right treatment. The following doctors resort to the help: hematologist (selects intensive therapy for the patient), dermatologist (his therapeutic tactics are necessary for skin manifestations of the syndrome), neurologist (gets involved in the process when neurological disorders occur), cardiologist, pulmonologist.
Conclusion
It must be remembered that hypereosinophilic syndrome requires qualified medical attention. It is impossible to ignore such a pathology in any case, since with complications this often leads to death.
It is not worth hoping that everything will go away on your own - only a timely visit to a doctor and proper treatment can guarantee the success of therapeutic measures.