Agranulocytosis is what? Agranulocytosis in children and adults: symptoms, treatment and causes

Laboratory examination of blood is performed practically with each pathology. This is due to the informative nature of this analysis. By changes in individual blood parameters, one can judge the etiology of the disease, the severity of the pathological process. Information on the increase or decrease of certain specific plasma cells allows one to suspect specific disorders. For example, leukemia, various types of anemia. One of the laboratory signs is agranulocytosis. This is a syndrome that can occur independently or in combination with other changes in the composition of the blood. This sign indicates the presence of a serious infectious process.

Agranulocytosis is what?

Disease of the blood is the science of hematology. A violation, which indicates a severe pathological process, is agranulocytosis. This syndrome, characterized by a marked decrease in the level of white blood cells - leukocytes. They are necessary for fighting infectious agents that enter the body when infected.

The normal number of leukocytes in the blood is 4-9 * 10 ⁹ / l. An increase in the number of these cells indicates the development of an inflammatory focus. Lack of white blood cells can occur when the immune system is inadequate, hematopoiesis is oppressed, intoxication. Agranulocytosis is a laboratory syndrome characterized by a decrease in the level of leukocytes to 1 * 10 ⁹ / l and less (neutrophils - 0.75 * 10 ⁹ ). Because of the pronounced deficit of immune cells, various infectious processes of bacterial or viral etiology arise. To identify this syndrome, you need a clinical blood test.

The incidence of agranulocytosis

This hematologic syndrome occurs in the female and male populations. Congenital agranulocytosis is less frequent, in comparison with the acquired disease. Similar changes in the cellular composition of the blood are more susceptible to middle-aged women (from 40 years). The frequency of occurrence of this laboratory test is different in different countries. On average, agranulocytosis is detected in 1 out of 1,200 people. A significant decrease in the number of leukocytes is more susceptible to Caucasians.

Causes of agranulocytosis

Why develop agranulocytosis? The causes of this pathological condition depend on the variety of the disease. Congenital agranulocytosis is due to a hereditary factor. The syndrome refers to genetically determined pathologies. The risk that a child born of a sick mother or father will have this syndrome is very high. The reasons for the acquired agranulocytosis include the following:

1. Autoimmune diseases. Similar pathologies are: systemic lupus erythematosus, scleroderma, rheumatoid arthritis. With these diseases, immune protection is directed not only to foreign particles, but also to the body's own cells.
2. Exposure to ionizing radiation. This factor leads to the inhibition of not only white, but also red hematopoietic growth.
3. Reception of cytotoxic drugs. These medications are indicated for rheumatological and oncological diseases. The disadvantage of cytotoxic therapy is that the drugs destroy normal cells.
4. Long-term use of antibiotics (for example, with tuberculosis), NSAIDs and antiparasitic drugs.
5. Severe course of infectious diseases. A sharp decrease in the number of neutrophils can be observed in viral hepatitis, measles, rubella, smallpox, influenza, etc.

It is worth noting that agranulocytosis is not an independent pathology. This syndrome can not develop without a specific reason. It is always associated with some kind of pathological effect.

Mechanism of development of agranulocytosis

Neutrophils belong to the protective cells of the blood. They are formed in the bone marrow and are released in large quantities continuously. These cells account for about 70% of the total number of leukocytes. With insufficient development of these blood elements, neutropenia develops. Agranulocytosis is considered a neglected form of this condition. Congenital deficiency of neutrophils, as well as the total number of leukocytes, arises from the presence of a pathological gene.

As a result of exposure to exogenous factors, the production of white blood cells decreases or completely ceases. Another mechanism for the development of the syndrome is the production of antibodies to neutrophils. Thus, agranulocytosis arises. The blood tests will change almost immediately. After all, the life span of neutrophils is rather short. It is from 2 to 3 days.

Classification of pathological conditions

Depending on the cause and pathogenetic mechanism, several classifications of agranulocytosis are distinguished. According to this, the congenital and acquired deficiency of neutrophils is isolated. In the first case, this hematologic syndrome occurs with the following genetic pathologies:

1. Granulomatous disease.
2. Family neutropenia.
3. Syndrome Shvakhman-Daemond.
4. Kostmann's disease.
5. Syndrome Shedyaka-Steinbrinka-Higashi.
6. Congenital aleukia.

The acquired deficiency of white blood cells is divided into autoimmune, myelotoxic and haptenic agranulocytosis. Each of these forms has its own development mechanism. On the course of the pathological process, acute conditions and chronic agranulocytosis are isolated. Also, the classification is based on the degree of severity of the syndrome.

Description of immune agranulocytosis

Immune agranulocytosis is associated with the pathological effect of antibodies on blood cells. It can be caused by both endo- and exogenous mechanism. In the first case, the disease occurs due to autoimmune aggression. This condition leads to systemic pathologies of connective tissue. Cells of the immune system do not change in composition, however they are "reprogrammed". As a result, they begin to counteract the body's own tissues. Granulocytes quickly ripen and self-destruct. In this case, the exogenous factors do not matter. Syndrome of immune agranulocytosis occurs in pathologies such as systemic lupus erythematosus and rheumatoid arthritis. In this case, the deficit of neutrophils is combined with the deficiency of other blood cells.

Another type of disease that occurs against the backdrop of immune processes is the hapten agranulocytosis. With this form of pathological process, antibodies to neutrophils also form. The difference is that they are not developed independently, but after taking some medications - haptens. Repeated intake of these substances causes an immune reaction, expressed in the destruction of granulocytes. Some antibacterial drugs, hypoglycemic and anti-inflammatory agents, barbiturates can act as haptens.

Description of myelotoxic agranulocytosis

Myelotoxic agranulocytosis develops as a result of exogenous effects. It is always combined with erythro- and thrombocytopenia. In most cases, the myelotoxic variant of the disease is associated with the effects of chemotherapy and ionizing radiation. These factors lead to oppression of hemopoiesis at the initial levels of its development. Cytostatic drugs that cause such a reaction include cyclophosphamide, fluorouracil, mercaptopurine, etc. Oppression of progenitor cells of hemopoiesis leads to a decrease in the number of granulocytes in the bone marrow. This is due to a disruption in the maturation of blood sprouts.

In addition to exogenous effects, deficiency of hematopoiesis cells develops in some types of anemia and leukemia. These causes are often due to agranulocytosis in children. Often, hematological changes are hereditary. Examples are hemolytic and aplastic anemia, Fanconi syndrome. Acute and chronic myeloblastic leukemias develop both in childhood and among adults.

Agranulocytosis: symptoms of the disease

The incidence of granulocytes is accompanied by severe clinical manifestations. They can be different, depending on the underlying disease that led to a neutrophil deficiency. How is agranulocytosis manifested? Symptoms of pathology:

1. Pronounced general weakness.
2. High fever.
3. Lymphadenopathy.
4. Hepatosplenomegaly.
5. Necrotic changes in mucous membranes.
6. Hemorrhagic syndrome.

Hapten agranulocytosis, in contrast to other forms, is characterized by acute course. Patients rapidly deteriorate, feel fever, ulcers appear in the mouth. Damage to the gums, tonsils, tongue, soft palate progresses, leading to tissue necrosis. This is accompanied by pain syndrome, inability to swallow, increased salivation.

With myelotoxic variant of agranulocytosis, bleeding often develops. They are associated not only with ulceration of the mucous membranes, but also with a deficiency of platelets. There are nasal, gingival, uterine, gastrointestinal hemorrhages. On the skin, hematomas appear. Due to ulcerative-necrotic processes in the intestine, diarrhea and abdominal pain are noted. As a result of blood aspiration, hemorrhagic pneumonia develops.

Changes in the analysis of agranulocytosis

Often the development of agranulocytosis occurs in patients with oncological, systemic and severe infectious diseases. To suspect this syndrome is possible by such signs as high fever, weakness and the appearance of ulcers in the oral cavity. It is more difficult to diagnose congenital agranulocytosis. The analysis of blood should be performed with the expressed paleness of the skin in the child, hemorrhagic manifestations. The study showed a decrease in leukocytes (less than 1 * 10 ⁹ / l). Often this is combined with anemia and thrombocytopenia.

To find out why the hematologic syndrome developed, perform an immunogram and a bone marrow puncture. To diagnose complications caused by a deficit of granulocytes, instrumental studies are carried out. Among them - chest X-ray, colonoscopy. Also, specialist consultations are required - an otolaryngologist, an oncologist, a dentist, a rheumatologist and a gastroenterologist.

Methods of treatment of pathology

Patient hospitalization is necessary if agranulocytosis is detected. Treatment consists in treatment of ulcerative-necrotic surfaces with antiseptic agents, ointment preparations. That the patient was not subjected to secondary infection, he is placed in an isolation ward. In case of damage to the intestinal mucosa, nutrition is parenteral.

Medications for the treatment of agranulocytosis

To prevent the development of the infectious process (pneumonia, sepsis), prescribe antibiotics and antifungal medicines. Intravenously injected blood products, in particular, leukocyte mass. At immune agranulocytosis hormonal medicines are shown. These include preparations "Hydrocortisone", "Prednisolone". If the syndrome is caused by oppression of hemopoiesis, stimulants are administered. Among them, the solution is "Granocyte", "Leucogen", "Virudan". Treatment is carried out in the department of hematology.

Prevention of agranulocytosis in children and adults

Prevention of congenital agranulocytosis involves early detection of the disease. For this purpose, children are given a clinical blood test every year. Prevention measures include monitoring patients taking cytotoxic drugs, anti-tuberculosis hypoglycemic agents. If a patient has previously had a hematological reaction to the administration of certain drugs, then these medicines can not be re-appointed.

Prognosis for life in agranulocytosis

The prognosis depends on the cause of the syndrome. The most dangerous form is the hapten agranulocytosis, since it rapidly develops tissue necrosis. Autoimmune and myelotoxic lesions can be treated with replacement therapy. An unfavorable prognosis is noted in the development of complications of the underlying pathology. Among them - massive bleeding, perforation of the intestinal wall, hemorrhagic pneumonia, sepsis.