Agranulocytosis - symptoms in children and adults, first signs and manifestations. What is agranulocytosis, and how to treat it? Drug-induced agranulocytosis

Agranulocytosis is a clinical and laboratory syndrome, the main manifestation of which is a sharp decrease or complete absence of neutrophil granulocytes in the peripheral blood, which is accompanied by an increase in the body’s susceptibility to fungal and bacterial infections.

Granulocytes are the most numerous group of leukocytes, in which, when stained, specific granules (granularity) become visible in the cytoplasm. These cells are myeloid and are produced in the bone marrow. Granulocytes take an active part in protecting the body from infections: when an infectious agent penetrates the tissue, they migrate from the bloodstream through the capillary walls and rush to the site of inflammation, here they absorb bacteria or fungi, and then destroy them with their enzymes. This process leads to the formation of a local inflammatory response.

With agranulocytosis, the body is not able to resist infection, which often becomes a factor in the occurrence of purulent-septic complications.

In men, agranulocytosis is diagnosed 2-3 times less often than in women; People over 40 years of age are most susceptible to it.

Causes

In the autoimmune form of the syndrome, a certain malfunction occurs in the functioning of the immune system, as a result of which it produces antibodies (so-called autoantibodies) that attack granulocytes, thereby causing their death. Autoimmune agranulocytosis can occur against the background of the following diseases:

• autoimmune thyroiditis;
• systemic lupus erythematosus;
• rheumatoid arthritis and other types of collagenosis.

Agranulocytosis is also of an immune nature, developing as a complication of infectious diseases, in particular:

• polio;
• viral hepatitis;
• typhoid fever;
• yellow fever;
• malaria;
• infectious mononucleosis;
• flu

With agranulocytosis, the body is not able to resist infection, which often becomes a factor in the occurrence of purulent-septic complications.

Currently, the hapten form of agranulocytosis is often diagnosed. Haptens are called chemical substances, the molecular weight of which does not exceed 10,000 Da. These include many medications. Haptens themselves do not have immunogenicity and acquire this property only after combining with antibodies. The resulting compounds can have a toxic effect on granulocytes, causing their death. Once developed, hapten agranulocytosis will be repeated every time the same hapten (drug) is taken. The most common causes of hapten agranulocytosis are the drugs Diacarb, Amidopyrine, Antipyrin, Analgin, Aspirin, Isoniazid, Meprobamate, Butadione, Phenacetin, Indomethacin, Novocainamide, Levamisole, Methicillin, Bactrim and other sulfonamides, Leponex, Chloroquine, barbiturates.

Myelotoxic agranulocytosis occurs as a result of a disruption of the process of myelopoiesis that occurs in the bone marrow and is associated with suppression of the production of progenitor cells. The development of this form is associated with the effect on the body of cytostatic agents, ionizing radiation and certain medications (Penicillin, Gentamicin, Streptomycin, Levomycetin, Aminazine, Colchicine). With myelotoxic agranulocytosis in the blood, the number of not only granulocytes, but also platelets, reticulocytes, and lymphocytes decreases, which is why this condition is called a cytotoxic disease.

In order to reduce the risk of infectious and inflammatory diseases, treatment of patients with confirmed agranulocytosis is carried out in the aseptic block of the hematology department.

Forms

Agranulocytosis can be congenital or acquired. Congenital is associated with genetic factors and is extremely rare.

Acquired forms of agranulocytosis are detected with a frequency of 1 case per 1300 people. It was described above that, depending on the characteristics of the pathological mechanism underlying the death of granulocytes, the following types are distinguished:

• myelotoxic (cytotoxic disease);
• autoimmune;
• haptenic (medicinal).

A genuine (idiopathic) form is also known, in which the cause of the development of agranulocytosis cannot be established.

According to the nature of the course, agranulocytosis can be acute or chronic.

Signs

The first symptoms of agranulocytosis are:

• severe weakness;
• pale skin;
• joint pain;
• increased sweating;
• elevated body temperature (up to 39–40 °C).

Ulcerative-necrotic lesions of the mucous membrane are characteristic oral cavity and pharynx, which may take the form of the following diseases:

• angina;
• pharyngitis;
• stomatitis;
• gingivitis;
• necrotization of the hard and soft palate, uvula.

These processes are accompanied by spasm of the masticatory muscles, difficulty swallowing, sore throat, and increased salivation.

With agranulocytosis, the spleen and liver become enlarged, and regional lymphadenitis develops.

Features of the course of myelotoxic agranulocytosis

Myelotoxic agranulocytosis, in addition to the symptoms listed above, is characterized by a moderately severe hemorrhagic syndrome:

• formation of hematomas;
• nosebleeds;
• increased bleeding of gums;
• hematuria;
• vomiting mixed with blood or in the form of “coffee grounds”;
• visible scarlet blood in the stool or black, tarry stool (melena).

Diagnostics

To confirm agranulocytosis, a complete blood count and bone marrow puncture are performed.

A general blood test determines severe leukopenia, in which the total number of leukocytes does not exceed 1–2 x 10 9 / L (normal is 4–9 x 10 9 / L). In this case, either granulocytes are not detected at all, or their number turns out to be less than 0.75 x 10 9 / l (the norm is 47–75% of the total number of leukocytes).

When examining the cellular composition of the bone marrow, the following is revealed:

• decrease in the number of myelokaryocytes;
• increase in the number of megakaryocytes and plasma cells;
• impaired maturation and decreased number of neutrophilic germ cells.

To confirm autoimmune agranulocytosis, the presence of antineutrophil antibodies is determined.

If agranulocytosis is detected, the patient should be consulted by an otolaryngologist and dentist. In addition, he needs to undergo a three-time blood test for sterility, a biochemical blood test and a chest x-ray.

Agranulocytosis requires differential diagnosis with hypoplastic anemia, acute leukemia, and HIV infection.

Agranulocytosis can be congenital and acquired, and the first form is an extremely rare phenomenon and is caused by hereditary factors.

Treatment

Treatment of patients with confirmed agranulocytosis is carried out in the aseptic unit of the hematology department, which significantly reduces the risk of infectious and inflammatory complications. First of all, it is necessary to identify and eliminate the cause of agranulocytosis, for example, discontinue the cytostatic drug.

For immune agranulocytosis, high doses of glucocorticoid hormones are prescribed and plasmapheresis is performed. Transfusions of leukocyte mass, intravenous administration of antistaphylococcal plasma and immunoglobulin are indicated. To increase the production of granulocytes in the bone marrow, you need to take leukopoiesis stimulants.

Regular mouth rinses with antiseptic solutions are also prescribed.

If necrotizing enteropathy occurs, patients are transferred to parenteral nutrition ( nutrients administered by intravenous infusion of solutions containing amino acids, glucose, electrolytes, etc.).

To prevent secondary infection, antibiotics and antifungal drugs are used.

The prognosis worsens significantly with the development of severe septic conditions, as well as in the case of repeated episodes of hapten agranulocytosis.

Prevention

Without a doctor's prescription and careful hematological monitoring, it is unacceptable to take medications that have a myelotoxic effect.

If an episode of hapten agranulocytosis is recorded, you should not subsequently take the drug that caused its development.

Consequences and complications

The most common complications of agranulocytosis are:

• pneumonia;
• hemorrhage;
• hepatitis;
• sepsis;
• mediastinitis;
• peritonitis.

The prognosis worsens significantly with the development of severe septic conditions, as well as in the case of repeated episodes of hapten agranulocytosis.

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Agranulocytosis

Agranulocytosis (agranulocytosis; Greek negative prefix a-, Latin granulum-grain and Greek kytos - cell; former names - aleukia, aneutrophilosis, granulophthisis) is a syndrome characterized by a sharp decrease in the number or absence of neutrophilic granulocytes in the peripheral blood. Agranulocytosis does not have clear boundaries with granulocytopenia, a clinically asymptomatic condition, therefore agranulocytosis is conventionally defined as a blood content of less than 750 granulocytes per 1 μl [Andrews, 1969] or a total number of leukocytes less than 1000 per 1 μl.

Agranulocytosis was first described in 1922 by W. Schultz. At the same time, Friedemann described agranulocytic tonsillitis. With the widespread use of cytotoxic therapy (drugs and radiation), as well as a large arsenal of new agents, the incidence of agranulocytosis has increased significantly.

Etiology and pathogenesis.

According to the mechanism of occurrence, agranulocytosis can be myelotoxic and immune.

Myelotoxic agranulocytosis

Myelotoxic agranulocytosis occurs as a result of suppression of the growth of prestage granulocytes in the bone marrow, including stem cells. In this regard, there is a decrease in the blood not only of granulocytes, but also of platelets, reticulocytes and lymphocytes. Myelotoxic agranulocytosis can develop as a result of exposure of the body to ionizing radiation, chemical compounds with cytostatic properties (antitumor drugs, benzene and others), waste products of a Fusarium type fungus that multiplies in overwintered grain.

Immune agranulocytosis

Immune agranulocytosis develops as a result of accelerated death of granulocytes under the influence of anti-leukocyte antibodies; stem cells are not affected. Anti-leukocyte antibodies are formed under the influence of medications that can play the role of haptens. With repeated administration of such a medication, agglutination of leukocytes appears.

The development of immune agranulocytosis depends little on the dose of the drug; the most important role in its occurrence is played by the unusual sensitivity of the body. On the contrary, in myelotoxic agranulocytosis, the decisive role is played by the magnitude of the damaging effect. Among the drugs that cause immune agranulocytosis, the main place belongs to amidopyrine. Immune agranulocytosis can also be caused by butadione, phenacetin, atophan, analgin, diacarb, barbamyl, sulfonamides, PAS, tubazide, ethoxide, streptomycin, pipolfen and some other drugs. Long-term use of the hapten drug can cause the destruction of not only mature granulocytes, but also myelocytes and promyelocytes. The development of autoimmune agranulocytosis, more often leukopenia, is observed with collagenosis (especially disseminated lupus erythematosus, rheumatoid polyarthritis), as well as with some infections.

Agranulocytosis occupies a special place in systemic lesions of the hematopoietic apparatus - leukemia, hypoplastic anemia, as well as in metastases to the bone marrow of a cancerous tumor and sarcoma.

Pathological anatomy.

Pathological signs of agranulocytosis are necrotic-ulcerative changes, most often found in the oral cavity and pharynx. The tonsils are enlarged, loose, gray-dirty in appearance, with fibrinous deposits and ulcerations. In the area of ​​the soft and hard palate, foci of necrosis are found, sometimes with perforation of the soft palate.

Necrotic changes are detected in the skin, at injection sites, in the perineal area, and around the anus. Foci of necrosis are described in the conjunctival sac, in the mucous membrane of the larynx, esophagus and stomach. With the development of necrosis in the mucous membrane of the small or large intestine, including the appendix, intestinal bleeding and perforation are observed. Necrotic ulcers can be in the wall of the bladder, in the genitals, especially in the vaginal wall, as well as in the tissue of the liver and other organs. Microscopic examination shows that there are no neutrophilic leukocytes in areas of necrosis. The demarcation strip around the necrosis is not detected; lymphohistiocytic and plasma cell accumulations can be seen near the areas of necrosis.

Pneumonia is fibrinous-hemorrhagic in nature. In this case, fibrinous deposits are located on the pleura. In the area of ​​pneumonia, areas of tissue decay (gangrene) may be detected. Microscopically, desquamated epithelial cells, bacteria, yeast cells and their mycelium are visible in the lumens of the alveoli. Lymphatic nodes are usually not enlarged. With necrotic changes in the oral cavity, a slight increase in cervical and submandibular lymph nodes may be observed. Their microscopic structure is relatively preserved. In the cortex and especially in the area of ​​the medullary cords, a large number of plasma cells are detected. Proliferation and swelling of reticuloendothelial cells in the sinuses is sharply expressed. The spleen is often unchanged. The spleen tissue is of a softish consistency; on section there is a pink-gray pulp with a large scraping. Microscopic examination reveals a uniform decrease in the number of red pulp cells of the spleen. The bone marrow of flat bones is macroscopically more common than usual, somewhat dry, but there may be various foci of hemorrhage - from small to extensive; in the lower and middle third of the tubular bones, the bone marrow is fatty. Microscopically, small foci of resorption of bone beams with the formation of small lacunae are revealed. Osteoblast proliferation may be observed in areas of bone resorption. The ratio of adipose and hematopoietic tissue is different. More often there is a decrease in the number of hematopoietic cells and an increase in the number of fat cells in the bone marrow. In the cellular composition, attention is drawn to a sharp decrease in the number of young, band and segmented granulocytes. There may be some predominance of young forms of granulocytes. Megakaryocytes and red row cells are usually preserved. In the most severe course of agranulocytosis, the bone marrow picture is the same as in hypoplastic anemia.

Clinical picture.

Myelotoxic agranulocytosis begins gradually: without any subjective signs. Immune agranulocytosis can clinically have different variants depending on the factor that caused it. Agranulocytosis against the background of collagenosis develops gradually and is characterized by persistent progression. The onset of drug-induced immune agranulocytosis is acute in most cases.

The first manifestations of any agranulocytosis are fever, stomatitis, and sore throat. With myelotoxic syndrome, a moderately severe hemorrhagic syndrome is also usually observed (bruising and bleeding gums, nosebleeds): severe hemorrhagic syndrome is an uncommon complication of agranulocytosis. Damage to the mucous membranes (necrosis and thrush) of the oral cavity and gastrointestinal tract is the most consistent sign of agranulocytosis. In myelotoxic agranulocytosis, it is due to the fact that, on the one hand, the disappearance of granulocytes makes microbial invasion possible, on the other hand, the suppression of mitoses of epithelial cells of the mucous membrane by cytostatic factors violates its integrity.

In the peripheral blood, the number of all forms of leukocytes decreases (often up to hundreds of cells in 1 μl), as well as platelets and reticulocytes. The number of plasma cells is usually increased. There may be anemia. Sometimes granulocytes disappear completely. Leukopenia in immune agranulocytosis is moderate - 1000-2000 cells in 1 μl, but the number of granulocytes, as a rule, decreases to zero; thrombocytopenia is absent. Anti-leukocyte antibodies are detected in the serum.

At the height of myelotoxic agranulocytosis, both granulocytic elements, erythronormoblasts, and megakaryocytes almost completely disappear in the bone marrow; lymphoid, reticular and plasma cells are preserved. 2-3 days before recovery from the state of agranulocytosis, prompelocytes and single normoblasts appear in the bone marrow in huge numbers. In peripheral blood, the first sign of activation of hematopoiesis is the detection of young elements - myelocytes and metamyelocytes, sometimes plasma cells. Often 2-3 days before the appearance of granulocytes, the number of platelets and reticulocytes increases.

In the bone marrow with immune agranulocytosis, a decrease in cellular elements is observed exclusively due to the granulocytic germ. Recovery from immune agranulocytosis is characterized by the appearance in the peripheral blood of young cells - myelocytes, metamyelocytes, and sometimes promyelocytes.

The duration of agranulocytosis varies and depends both on the degree of bone marrow damage and on the individual characteristics of the patient’s body.

Complications.

The most common are sepsis (often staphylococcal), intestinal perforation (usually the ileum, since it is more sensitive to cytostatic effects), mediastinitis, pneumonia, noma; less often - severe swelling of the intestinal mucosa with the formation of obstruction and peritonitis. A serious complication is acute epithelial hepatitis, which often develops after the elimination of agranulocytosis. The absence of granulocytes gives a unique character to the course of infectious complications - the absence of ulcers, the predominance of necrosis. Pneumonia occurs against the background of scanty physical data: dullness is barely noticeable, there may be no wheezing or pronounced bronchial breathing, only crepitus is heard over the affected area. X-ray changes are very scarce.

Diagnosis

The diagnosis is made on the basis of anamnestic data, a characteristic clinical picture, data from a study of peripheral blood and bone marrow puncture. The diagnosis of immune agranulocytosis can be confirmed by serological studies: detection of anti-leukocyte antibodies (see Blood groups, leukocyte groups). Agranulocytosis must be differentiated from acute leukemia in the aleukemic stage and hypoplastic anemia. In the first case, the decisive factor in diagnosis is the study of bone marrow puncture, in the second - an indication of the sudden development of agranulocytosis due to its immune nature or information about long-term use of drugs with myelotoxic action (sometimes interrupted several weeks before the development of agranulocytosis). Observation of the patient allows us to come to a final conclusion about the nature of bone marrow devastation.

The prognosis in most cases of mnelotoxic agranulocytosis under aseptic conditions and timely powerful antibiotic therapy is favorable. Necrotic enteropathy, as well as severe septic complications, sharply worsen the prognosis. The prognosis for immune agranulocytosis of drug origin in the conditions of timely discontinuation of the hapten drug, steroid hormone therapy and subject to all rules for the management of such patients is in most cases favorable.

Treatment of agranulocytosis

Treatment of agranulocytosis of any origin requires immediate elimination of the cause that caused it: a cytostatic drug, ionizing radiation, a hapten drug that provoked an immune conflict, and so on. Therapy for agranulocytosis, which complicates the course of rheumatoid arthritis or disseminated lupus erythematosus, should first of all be directed against the underlying disease.

In myelotoxic agranulocytosis, steroid hormones are not indicated. When the number of leukocytes decreases to 50-200 cells in 1 μl, replacement therapy is necessary - transfusion of leukocyte concentrate. More than 15-20 billion cells are transfused at the same time. In cancer patients, a good effect was observed when using leukocyte mass obtained from patients with chronic myeloid leukemia in the advanced stage of the disease. Bone marrow transplantation is used only in acute total irradiation at doses exceeding 600 rads per bone marrow. Along with the use of pathogenetic agents, symptomatic therapy is also used: in case of persistent hyperthermia up to 39-40° with myelotoxic agranulocytosis (but not immune!), patients are given analgin up to 2-3 g per day or acetylsalicylic acid - 2 g per day.

In the treatment of immune agranulocytosis, the decisive role belongs to prednisolone at a dose of 1-1.5 mg per 1 kg of weight or its analogues in adequate doses, and in the absence of effect - 2-3 times more. In case of severe damage to the mucous membranes, the drug is administered parenterally. The course is 7-10 days or until agranulocytosis is eliminated. Replacement therapy with leukocyte transfusions is indicated only for infectious complications.

To prevent infection, patients must be placed in boxes or isolation rooms where aseptic conditions are created. Prevention of infectious complications with antibiotics is necessary when the number of granulocytes drops to 750 in 1 μl. The non-absorbable antibiotic neomycin is prescribed orally up to 2-3 g per day (against E. coli), polymyxin B 150-200 mg per day (against Pseudomonas aeruginosa). In addition, a broad-spectrum antibiotic is prescribed - garamycin intramuscularly 40-80 mg 2-3 times a day, or oxacillin 4-6 g per day orally, or oletethrin up to 2 g per day orally. Be sure to take nystatin up to 10,000,000 - 15,000,000 units per day. For systematic rinsing of the mouth, use a solution of levorin 1: 500. Ulcers on the mucous membrane of the mouth and lips are lubricated with sea buckthorn oil. With the development of staphylococcal sepsis, the administration of antistaphylococcal gammaglobulin and antistaphylococcal plasma is indicated. If diarrhea, bloating, or persistent severe pain in the abdomen occurs, the patient must be transferred to parenteral nutrition, and surgical supervision becomes necessary. Perforation of intestinal ulcers, characterized by mild signs of irritation of the peritoneum, increasing bloating, loss of intestinal motility, and the appearance of effusion in the abdominal cavity, makes urgent surgical intervention necessary.

Prevention

Prevention of myelotoxic agranulocytosis consists of careful hematological monitoring during therapy with cytostatic drugs. If the number of leukocytes decreases to 1000-1500 per 1 μl or their rapid decrease, it is necessary to stop cytostatic therapy or take a break in treatment. However, with leukopenic variants of acute leukemia, therapy must be carried out with small or medium doses of cytostatic drugs, despite the low numbers of leukocytes, focusing on their dynamics and platelet levels. A persistent decrease in the latter indicates the need for a break in cytostatic treatment. Prevention of immune agranulocytosis is aimed at avoiding repeated use of drugs that previously turned out to be the cause of agranulocytosis in a given patient.

Agranulocytosis in children.

The same mechanisms play a role in the development of agranulocytosis in children as in adults. The clinical picture of mnelotoxic and immune agranulocytosis corresponds to that in adults.

In children in early age Relatively rarely, peculiar neutropenias occur, which, in terms of clinical features and complications, largely resemble the picture of agranulocytosis. There are two main types of neutropenia: constant and intermittent.

Permanent neutropenia, described by Kostmann (R. Kostmann, 1956), is characterized by the occurrence in early childhood of recurring purulent diseases: abscesses on the skin, in the lungs, otitis media, and the like. With the appearance of teeth, amphodontosis develops, leading to early tooth loss. Neutropenia is observed in the blood with a normal or slightly reduced number of leukocytes. Red blood and platelet counts are unchanged. This form of the disease is inherited in a recessive manner. Isolated cases of a similar disease, which has a milder course and is dominantly inherited, have been described [Hitzig, 1959]. In the bone marrow, there is a stop in the maturation of neutrophils at the promyelocyte stage, and an increased percentage of monocytes and eosinophils.

Periodic neutropenia is characterized by regular rises in temperature, outbreaks of purulent processes, and the disappearance of mature neutrophils from the blood and bone marrow. The neutropenic period lasts several days, then the normal blood picture is restored. During the period of neutropenia, the picture of blood and bone marrow corresponds to that with a constant form. In most cases, the disease is inherited in a recessive manner [Page and co-workers, A. Videbaek], less often in a dominant manner.

Along with hereditary forms, transient congenital neutropenia [Stefanini (M. Stefanini)] has been described in infants whose mothers suffer from granulocytopenia.

Treatment of agranulocytosis in children follows the same rules as in adults. In the treatment of hereditary neutropenia, the most important role is played by antibiotics, which are prescribed in the periodic form 2-3 days before the expected crisis for the entire period of neutropenia. With persistent neutropenia, antibiotic therapy is carried out in connection with purulent complications. The leading place belongs to antistaphylococcal drugs, as well as broad-spectrum antibiotics: oxacillin, methicillin, zeporin and others. With the introduction of massive antibiotic therapy, mortality in the first years of life, the most dangerous period of the disease, sharply decreased; In the future, the disease proceeds somewhat milder, patients live to adulthood. In addition to general therapy, systematic sanitation of the oral cavity and gum care are carried out.

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The content of the article

Etiology of agranulocytosis

Treatment of agranulocytosis

Prevention of agranulocytosis

Autoimmune agranulocytosis

Hapten agranulocytosis

Etiology of hapten agranulocytosis

Pathogenesis of hapten agranulocytosis

Clinic of hapten agranulocytosis

Treatment of hapten agranulocytosis

Myelotoxic arganulocytosis

Etiology and pathogenesis of myelotoxic agranulocytosis

Clinic of myelotoxic agranulocytosis

Treatment

Agranulocytosis is characterized by a critically reduced granulocyte count. These elements are representatives of one of the main fractions of the leukocyte series.

This is a very severe blood pathology, the diagnosis of which is made if granulocytes are not detected or there are less than 0.75 * 109 / μl, and the number of leukocytes is no more than 1.5 * 109 cells per μl. The symptoms of agranulocytosis depend on the form of the disease, which we will consider further.

Symptoms of myelotoxic agranulocytosis

Myelotoxic agranulocytosis is a consequence of a process occurring in the bone marrow. It inhibits the growth of prestage granulocytes. This also applies to stem cells. Therefore, the number of granulocytes and reticulocytes, platelets and lymphocytes in the blood decreases.

In the blood of a sick person, even before the symptoms of the disease appear, the number of platelets, leukocytes and reticulocytes decreases. Leukopenia may develop. Neutrophil granulocytes in the peripheral blood decrease sharply, they can completely disappear. As the bone marrow and peripheral blood undergo changes, symptoms such as:

• angina;
• bleeding gums;
• stomatitis;
• bleeding under the skin.

Sometimes only the mucous membrane of the larynx and mouth may be affected. This occurs due to the fact that the body’s defenses weaken, so pathogenic microorganisms are activated. Also, antitumor drugs affect proliferating cells and mucous digestive channels. Such changes may continue for more than a month.

Symptoms of immune agranulocytosis

Immune agranulocytosis is characterized by a reduced number of granular leukocytes in the peripheral blood.

There are two forms of this disease:

1. Hapten agranulocytosis. This form appears as a result of antibodies that appear in the blood against granulocytes. The antigen reaction is as follows: the antibody acts on a hapten, which is medicines. That's why clinical picture varies depending on the drug that caused the disease. Drug-induced agranulocytosis has an acute onset. In this case, the body temperature rises to 39 degrees. Sore throat, stomatitis, and sometimes candidiasis of the oral mucosa and esophagus also appear. If the case is very severe or a relapse occurs, the disease is complicated by necrotic enteropania. Immune agranulocytosis, which develops in this form, can be easily determined using laboratory blood tests.
2. Autoimmune agranulocytosis. In case of systemic connective tissue diseases, leukemia and some other ailments, this form proceeds slowly and its course is quite long.

It is worth noting that immune agranulocytosis is more common in women than in men. This form is less common in children.

General symptoms of agranulocytosis

Regardless of the form of the disease, some symptoms can be identified that indicate that treatment for agranulocytosis should be started. The fact is that leukocytes restrain the influence of microflora, thereby preventing it from multiplying much.

This function is mainly assigned to granulocytes. If agranulocytosis develops, microorganisms begin to multiply uncontrollably, and this leads to infectious complications.

First of all, this greatly affects the oral cavity, so the following diseases develop:

• tonsillitis;
• infectious stomatitis;
• pingivitis;
• pharyngitis.

The affected mucous membrane takes on a dirty gray coating, and bacteria multiply underneath it. In addition, the mucous membrane is abundantly supplied with blood, which allows bacterial toxins to actively enter the general bloodstream. This leads to severe intoxication, clinically manifested by high fever. In this case, the temperature reaches forty degrees and even higher. Nausea, weakness and headache are also observed.

Agranulocytosis manifests itself as another characteristic symptom - the rapid formation of ulcers. Necrotization of tissue spreads to adjacent surfaces and can acquire the character of a noma, the so-called water cancer. Similar processes spread to the gastrointestinal tract, where specific ulcerative-necrotic enteropathy develops. It is characterized by the formation of necrosis of the stomach, esophagus and intestines.

This situation is manifested by cramping severe pain in the abdomen. Diarrhea and vomiting appear. If necrotic lesions of the digestive tract are deep, severe bleeding begins, which threatens the patient’s life, so treatment of agranulocytosis should be started as early as possible. Jaundice may develop.

In addition, agranulocytosis can cause pneumonia, which can lead to the formation of abscesses and gangrene. The process can spread to neighboring organs. Lung damage makes itself felt by shortness of breath and cough. If the process spreads to the pleura, the person experiences chest pain while breathing. If the disease is severe, acute respiratory failure develops.

Rarely, the infectious process can begin from below and spread genitourinary organs. Such patients complain of pain during urination, in the lower abdomen, and so on. Women may notice abnormal discharge. Protein may be excreted in the urine, indicating albuminuria. This occurs due to intoxication, which leads to venous and arterial hypotension.

Symptoms of agranulocytosis in children

Agranulocytosis is very rare in children. However, almost all cases are characterized by hypoplastic anemia. In addition, with agranulocytosis, infectious diseases develop, which are accompanied by the following symptoms:

• fever;
• general weakness;
• increased heart rate;
• dyspnea;
• heavy sweating.

The disease can occur in combination with pneumonia, enlargement of the liver, spleen and lymph nodes, stomatitis, sore throat, and so on. As you can see, the symptoms in children are very similar to the signs of the same disease in adults. Symptoms should not be allowed to go unmonitored in any person. They are subject to treatment, which will avoid complications of insidious agranulocytosis.

There are a large number of different blood diseases. Among them is agranulocytosis. We must understand that this is not an independent disease, but a syndrome. That is, it is a sign or complication of some disease.

Blood structure

First, a little about the structure of human blood. Liquid tissue of the body consists of intercellular substance (plasma) and cells.

Blood cells are:

1. Red blood cells or red blood cells.
2. Leukocytes, white blood cells that protect the body from disease.

Leukocytes are divided into two types:

• Granulocytes. There are only three types of them: neutrophils, eosinophils and basophils.
• Agranulocytes. These are lymphocytes and macrophages.

1. Platelets. Small plates that help stop bleeding by participating in.

This is just a short list of the main cell types. It is necessary to understand what agranulocytosis is.

Agranulocytosis is a clinical and immunological syndrome characterized by a sharp (less than 0.75·109/l) decrease or complete disappearance of granulocytes in the blood, with a total of less than 1·109/l.

Agranulocytosis is a white blood disease. That is, there is an impact on the protective blood cells - leukocytes. With this disease, there are few granulocytes (a is the Latin prefix of negation. That is, agranulocytosis - no granulocytes).

The entire syndrome was divided into types, depending on the cause of agranulocytosis:

• immune agranulocytosis. It arises due to incorrectness.
• (pathological) reaction of the human immune system to its own blood cells.
• myelotoxic agranulocytosis is associated with an effect on the formation of granulocytes in the bone marrow.
• Genuine agranulocytosis or one for the development of which they could not find a reason.

In the case of an immune nature, the cause of destruction is antibodies (AT) to mature granulocytes or their precursors.

There are diseases due to which this condition appears:

• rheumatoid arthritis;

These diseases trigger an autoimmune mechanism for the production of antibodies to leukocytes. The hapten mechanism is activated when hypersensitivity body to certain drugs:

• amidopyrine;
• butadione;
• aminazine;
• indomethacin;
• delagil;
• chloramphenicol;
• sulfonamides;
• tuberculostatic drugs.

Myelotoxic agranulocytosis occurs due to the suppression of granulocyte development in the bone marrow by harmful effects on stem cells or myelopoiesis progenitor cells.

The causes of myelotoxic agranulocytosis can be:

• cytostatic drugs. They are prescribed during chemotherapy for cancer patients.
• ionizing radiation.
• some viruses (Epstein-Barr, rubella virus).

With genuine agranulocytosis, the cause cannot be found.

Symptoms

Clinical manifestations of agranulocytosis are associated with the development of infection.

Symptoms of agranulocytosis are primarily:

• poor general health. Severe weakness, pallor and sweating;
• elevated temperature (39º-40º), chills;
• the appearance of ulcers in the mouth, tonsils and soft palate. In this case, the person feels a sore throat, it is difficult for him to swallow, and drooling appears;
• pneumonia;
• ulcerative lesions of the small intestine. The patient feels bloated, has loose stools, and cramping abdominal pain.

In addition to the general manifestations of agranulocytosis, changes occur in the blood test:

• in humans, the total number of leukocytes sharply decreases;
• occurs, up to complete absence;
• relative lymphocytosis;

To confirm the presence of agranulocytosis in a person, a bone marrow test is prescribed.

After diagnosis, the next stage begins - treatment of agranulocytosis.

Treatment

Treatment of agranulocytosis is carried out in several stages, sequentially:

• The cause that caused the decrease in granulocytes in the blood is eliminated. But we must understand that sometimes it is impossible to completely remove the cause of agranulocytosis.
• The patient is transferred to a special sterile room. His immunity does not work and any accidental infection can lead to severe complications and death.
• A course of antibacterial drugs for agranulocytosis is prescribed. This allows you to stop the development of existing infections and prevent the emergence of new ones.
• Replacement therapy is carried out: transfusion.
• A course of hormonal therapy is prescribed.
• Measures are taken to stimulate the production of leukocytes.

In each case, to carry out effective treatment it is necessary to take into account the cause of agranulocytosis, the degree of severity, whether there are complications or not, what age the patient is (elderly or child), his general condition and much more. In addition to prescribing a course of necessary medications and placing the patient in a sterile environment, great attention is paid to proper nutrition.

for this disease it is prescribed by a doctor. It is based on:

• milk porridges (especially rice and rolled oats);
• jelly;
• lean meat and fish;
• dairy products.

Be sure to feed the patient in small portions, 5-6 times a day. Your doctor will help you create a more precise diet.

Doctors included agranulocytosis in children and agranulocytosis in pregnant women into a separate group.

Subtleties of agranulocytosis in children and pregnant women

Agranulocytosis is rare in children. Most often as a sign of tumor. Another feature: children are the only group of patients in whom congenital agranulocytosis occurs against the background of some hereditary diseases. Unfortunately, such patients do not live long. Otherwise, the tactics of treatment and diagnosis of agranulocytosis in young patients do not differ from those in adults.

Agranulocytosis in pregnancy is a condition in which the clear solution is to terminate the pregnancy.

After the diagnosis has been made and treatment prescribed, the question arises: how life-threatening is agranulocytosis, how long will the treatment last, and what will the result be?

Forecast

The cause of the syndrome plays a huge role in how successful the treatment will be and what the prognosis will be for agranulocytosis.

It is necessary to understand that the effectiveness of therapy against the background of cancer differs from the results of treatment of agranulocytosis caused by taking sulfonamides. In addition, it is important how severe the disease is. What complications of agranulocytosis appeared and whether they exist at all.

With a favorable course of the disease, recovery can be complete; in severe, complicated cases, mortality reaches 60%.

Possible complications

All complications of agranulocytosis are associated either with the condition itself or with the cause that caused it. Here is just a small list of factors aggravating the disease:

• development of infectious complications (pneumonia, necrotizing tonsillitis, sepsis)
• development of necrotizing enteropathy
• the production of antibodies to one’s own platelets and, as a consequence, the development of .

Any of these conditions is extremely life-threatening. Even with a completely correct diagnosis and absolutely adequate treatment, they can lead to the death of the patient.

Prevention

Prevention of agranulocytosis is related to the causes that cause it. If we are talking about hapten agranulocytosis, then as a preventative measure one should avoid taking medications that cause agranulocytosis. For all other species, this means constant monitoring of blood counts.