Agranulocytosis is a syndrome characterized by a sharp decrease in granulocytes in the blood (less than 1x109 / l, normally 6.8 × 109 / L), including neutrophils below 0.5-1x109 / l (normal 1.8-6 , 5 × 109 / L). Agranulocytosis is a variant of neutropenia, in which there is a decrease neutrophils in the blood. Neutrophils are part of the body's immune system, and this makes immunity weaker and more vulnerable to various infections.
This condition threatens with dangerous complications, and can be the cause of death. Even a common runny nose in such patients can be complicated by sepsis and coma, so agranulocytosis refers to a complex pathology and needs careful study and prevention.
Agranulocytosis is divided into immune, myelotoxic and genuine. Immune agranulocytosis appears by the formation of anti-granulocyte antibodies to granulocytes. And thanks to this in the blood, their quantity decreases. Immune agranulocytosis is divided into haptenic and autoimmune.
Agranulocytosis haptenovy manifested when using drugs (haptens), such as painkillers, antibiotics, tuberculosis drugs and others. With an autoimmune form, antibodies to granulocytes are produced and subsequently destroyed. This autoimmune process can be observed with various autoimmune diseases.
When myelotoxic agranulocytosis is inhibited the production of young cells of granulocytes in the bone marrow, and this leads to a reduced content of granulocytes in the blood. Genuine agranulocytosis is a form without an established cause.
Acute agranulocytosis is not symptomatically manifested, only in the laboratory. Patient's state of health worsens only when complications are attached, for example, severe infection (sepsis, necrotic tonsillitis , abscessing pneumonia, ulcerative stomatitis), which occurs due to reduced immunity.
In the first 2-3 days, with a reduced amount of granulocytes, the patient feels well and his body temperature is normal, but if the agranulocytosis lasts more than 6-7 days, the patient's condition worsens, dangerous infectious complications develop.
The patient's state of health is determined by the number of reduced granulocytes in the blood. At low numbers, the syndrome is more severe. With a small decrease in granulocytes, the infection may not develop if one maintains normal living conditions and excludes the infections that surround the patient. But if the patient is in the hospital, then even the slightest decrease in granulocytes in the blood can be complicated by severe infections.
The cause of infection, which complicates agranulocytosis, may be staphylococcus, E. coli, Pseudomonas aeruginosa and other bacteria. Symptomatically, this is manifested by an increase in temperature to 40-41 ° C, severe headache, vomiting, muscle pain, confusion, weight loss, redness of the skin, and a sharp drop in blood pressure. Also, small, dark foci can form on the skin that rise above the skin (more often with Pseudomonas aeruginosa).
The next terrible complication that occurs with agranulocytosis is necrotizing enteropathy. Symptomatically this is manifested by a frequent liquid stool, cramping pains in the abdomen, swelling, high fever, vomiting, general weakness. Sometimes ulcers are formed, which can be ruptured.
Thrombocytopenia and anemia are also considered complications of agranulocytosis. They are found most often in autoimmune agranulocytosis, since antibodies are formed to platelets and erythrocytes. Symptomatic thrombocytopenia is manifested by bleeding (hemorrhagic syndrome), and anemia - dizziness, weakness, headache, pallor of the skin, tachycardia (rapid heartbeat), etc.
Agranulocytosis in children
A child, this is an unusual organism, which is always more sensitive to all external favorable and unfavorable factors, and the response to these factors is twice that of an adult. Therefore, children should be more careful and circumspect. Any child's doctor, after suffering a child's illness (any infection, otitis, colds), should send a mother and child to give a general blood test to prevent the reduction of leukocytes (granulocytes, neutrophils).
The causes of the syndrome of agranulocytosis in children are similar to those in adults, but still have their own characteristics. In childhood, one of the frequent causes of agranulocytosis is neutropenia in children, or chronic child benign neutropenia. It often manifests itself up to one year and lasts from several months to several years. This form is completely safe for the baby, does not manifest itself symptomatically, and does not require special treatment.
Doctors believe that agranulocytosis in children is manifested due to the immaturity of the blood system, which ripens to about 5 years. The development of complications with this form of neutropenia is not observed and, usually, this state passes by itself. The only thing that needs to be added is that antibiotics are used early for children with agranulocytosis as a prophylaxis for colds, and it is recommended that mothers with children take up clinical check-ups with a pediatrician, a hematologist, and an allergist-immunologist.
Further agranulocytosis may occur when taking various medications, for example, such as antibiotics. Much less often agranulocytosis can occur with the development of malignant blood diseases. Of course, immediately there is an alarm and chemotherapy.
It is quite another matter when agranulocytosis in a child develops against the background of persistent, frequent infectious diseases, in which the number of neutrophils in the blood can decrease. And it can be seen clinically: the children appear sores on the mucous membrane of the mouth, pneumonia may develop, or the temperature will rise greatly. If you do not treat these complications, your baby may develop a septic shock. If a small amount of neutrophils persists, then a disease such as febrile neutropenia may occur. It is manifested by the following symptoms: increased weakness, high fever, tremor, excessive sweating, stomatitis, periodontitis, gingivitis , frequent fungal diseases. Treatment of this form consists, first of all, in the removal of infection and the treatment of diseases that led to the emergence and progression of agranulocytosis.
Further it is necessary to strengthen the immune system of the child by using immunostimulating drugs, vitamins, food supplements. With a very severe form of the disease, the child is completely isolated in a sterile room, where ultraviolet irradiation is constantly carried out and high sterility is maintained. We must always remember that agranulocytosis is easier to treat at an early stage, and therefore, when the first appearance of this ailment should immediately turn to the pediatrician, who will then send to the hematologist for advice.
Often, doctors argue whether vaccinations should be given to a child if he has leukocytes lowered. Here we must emphasize and say that according to Russian standards vaccination of the child is delayed if the number of neutrophils is greatly reduced, but according to European standards this is not a contraindication to vaccination and many doctors-professors agree with this.
Treatment of agranulocytosis is divided into several points. First, the factor that contributed to the development of agranulocytosis is eliminated. For example, medicines, diseases of the autoimmune nature, various infections. Further, it is necessary to create aseptic conditions for the patient. Patients with agranulocytosis are placed in sterile rooms, boxes, visiting patients with relatives and friends is limited. These measures are prophylactic, in order to prevent the development of infections that greatly affect the patient's health and, in the future, even lead to his death.
Also, preventive maintenance and treatment of infectious complications is carried out. Prevention is the use of antibiotics. They are not used if the amount of granulocytes is reduced slightly (1.5x109 / l), and if it is greatly reduced (1x109 / l), antibacterial drugs are used. Usually, two broad-spectrum antibiotics (acting on different microbes) in tablets are prescribed. If the neutrophils in the blood are below 1x109 / L, then two antibiotics are administered intravenously or intramuscularly. Usually, cephalosporins with aminoglycosides are used. If an infection has already been attached, two or three broad-spectrum antibiotics of the same groups (cephalosporins, aminoglycosides, and penicillins) are used. When the patient is treated with antibiotics, antifungal drugs (such as Nystatin, Levorin and others) are prescribed in parallel. The treatment is performed until the granulocyte counts are at the normal level.
The treatment also includes transfusion of leukocyte mass or thawed white blood cells 2-3 times a week. This procedure is used when the granulocytes in the blood are greatly reduced (they are not used for immune agranulocytosis). The leukocyte mass is selected taking into account the compatibility of the leukocytes of the patient with the HLA-antigen system. Transfusion of the leukocyte mass is contraindicated in immune agranulocytosis. It reduces the production of granulocytes in the bone marrow and inhibits the production of antibodies. Apply Prednisolone in a dose of 40 to 100 mg per day, to normal figures of granulocytes in the blood. Then the dose is slowly reduced.
It is necessary to use leukopoiesis stimulants in the treatment of agranulocytosis. These include Sodium Nucleinate, Leucogen, Pentoxyl. Treatment continues for 3-4 weeks. Everything depends on the condition of the patient. Also apply Molgrastmost, Leikomax.
When strong intoxication detoxification therapy is used (fever, pallor, vomiting, nausea, stool disorders, severe weakness, low blood pressure). Apply hemodez, glucose, isotonic sodium chloride solution or Ringer's solution. Transfusion of platelets can be used in cases of severe hemorrhagic syndrome, which is caused by acute thrombocytopenia. The platelets received from one donor are poured. You can also use gemostatics, which stop bleeding. These include Dicycinum, Aminocaproic acid, etc. If severe anemia develops, then transfusion of erythrocytes is used. To prevent complications after a transfusion, washed eritrotsites are often used.
The patient with agranulocytosis is weakened and "unarmed", since his "weapons" - granulocytes (neutrophils) are formed in small amounts. Agranulocytosis is a serious problem, to date, which is not yet fully understood by scientists, but many laboratories are working to prevent and treat this ailment.
Prevention of agranulocytosis is very important moment of this syndrome, and therefore it is necessary to always maintain the immune system with vitamins. In the summer it is fruits, vegetables, and in the winter - it's tableted vitamins, which can support the human immune system.