Agranulocytosis - a syndrome characterized by a sharp decrease of granulocytes in the blood (less than 1h109 / liter, normally 6,8 × 109 / L), and including neutrophils below0,5-1h109 / L (normal 1,8-6,5 × 109 / L).Agranulocytosis is a variant of neutropenia, wherein there is a reduction neutrophils in the blood.Neutrophils are part of the immune system, and this makes immunity more weak and vulnerable to various infections.
This state is in danger of complications and can cause death.Even the common cold in these patients may be complicated by sepsis, and coma, so agranulocytosis is a complex disease and needs careful study and prevention.
Agranulocytosis divided into immune, myelotoxic and genuinny.Immune agranulocytosis occurs through the formation of antibodies to antigranulotsitarnyh granulocytes.And thanks to their number in the blood decreases.Immune agranulocytosis divided into hapten, and autoimmune.
Agranulocytosis hapten appears when
When myelotoxic agranulocytosis inhibits the production of young cells of granulocytes in the bone marrow, and this leads to a decrease in the content of granulocytes in the blood.Genuinny agranulocytosis - a form without an established cause.
Acute agranulocytosis symptomatically manifested not only by laboratory tests.The patient's condition is getting worse only upon accession of complications, such as severe infection (sepsis, necrotic angina, pneumonia, abscess, ulcerative stomatitis), which arises in connection with lowered immunity.
In the first 2-3 days, with a reduced number of granulocytes, the patient feels well and his body temperature is normal, but if agranulocytosis lasts more than 6-7 days, the patient's condition worsens, threatening infectious complications develop.
state of health of the patient is determined by the number of granulocytes in the blood reduced.At low numbers syndrome is more severe.For a small decrease in granulocytes infection can not develop if to maintain normal living conditions and eliminate the infection, which surround the patient.But if the patient is in the hospital, even the slightest decrease of granulocytes in the blood may be complicated by severe infections.
cause of the infection, which complicates agranulocytosis, could be aureus, Escherichia coli, Pseudomonas aeruginosa and other bacteria.Symptomatic is seen raising the temperature to 40-41 ° C, severe headache, vomiting, muscle pain, confusion, weight loss, redness of the skin, a sharp drop in blood pressure.Also on the skin can form small, dark lesions, which rises above the skin (often with Pseudomonas aeruginosa).
next threatening complication that occurs when agranulocytosis is necrotic enteropathy.Symptomatic is manifested loose stools, cramping abdominal pain, bloating, fever, vomiting, general weakness.Sometimes ulcers are formed, which can rupture.
Thrombocytopenia and anemia are also considered complications of agranulocytosis.They occur most often in patients with autoimmune agranulocytosis, since the antibodies are formed in platelets and red blood cells.Symptomatic thrombocytopenia seen bleeding (hemorrhagic syndrome), and anemia - dizziness, weakness, headache, pale skin, tachycardia (rapid heartbeat), and so forth.
child is an extraordinary body, which is always more sensitive toall the favorable and unfavorable external factors, and responses to these factors it has two times more than that of an adult.Therefore, children need to be more careful and circumspect.Any pediatrician, after an illness of the child (any infection, otitis media, colds), due for preventive reduction of white blood cells (granulocytes, neutrophils) that mother and child on the surrender of the general analysis of blood.
causes of agranulocytosis syndrome in children are similar to those in adults, but still have their own characteristics.In children, a common cause of agranulocytosis is neutropenia in infants, children or chronic benign neutropenia.It often manifests itself up to one year and lasts for a few months to several years.This form is completely safe for the baby, symptomatically does not manifest itself, and does not require special treatment.
Doctors believe that agranulocytosis in children appears because of the immaturity of the blood system, which matures approximately to 5 years.The development of complications of neutropenia in this form are not observed, and usually this state passes its own.The only thing that we must add - early used antibiotics to children with agranulocytosis as a prevention for colds, and always recommend to mother and child stand on the dispensary registration to the pediatrician, hematologist, allergist-immunologist.
Next agranulocytosis may occur when taking different medications, such as antibiotics.More rarely, agranulocytosis may occur during the development of malignant diseases of the blood.Of course, here it is necessary to sound the alarm immediately and chemotherapy.
quite another thing when a child agranulocytosis developed against the backdrop of persistent, frequent infectious diseases, which can reduce the number of neutrophils in the blood.This is seen clinically: children have sores appear on the oral mucosa, may develop pneumonia or cranking up the temperature.If you do not treat these complications, the baby may develop septic shock.If a small number of neutrophils will be maintained, there may be a disease such as febrile neutropenia.It is manifested by the following symptoms: increased weakness, fever, tremors, sweating, stomatitis, periodontal disease, gingivitis, frequent fungal diseases.Treatment of this form is primarily to remove the infection and the treatment of diseases that led to the initiation and progression of agranulocytosis.
Further it is necessary to strengthen the child's immune system through the use of immune-boosting drugs, vitamins and nutritional supplements.In very severe form of the disease the child is completely isolated in a sterile room where ultraviolet irradiation is carried out continuously and maintain a high sterility.We must always remember that agranulocytosis easier to treat at an early stage and, therefore, at the first appearance of this disease should go directly to the children's pediatrician, who will then pass to a hematologist for advice.
Most doctors argue whether to raise the child vaccinated if it reduced white blood cells.Here it must be emphasized and said that according to Russian standards defer vaccination child, if the number of neutrophils greatly reduced, but by European standards it is not a contraindication to vaccination, and many doctors, professors agree.
Treatment of agranulocytosis is divided into several areas.At first, it eliminated the factor which contributed to the development of agranulocytosis.For example, medications, autoimmune diseases, various infections.Next, create aseptic conditions for the patient.Patients with agranulocytosis placed in sterile rooms, boxes, limited visiting sick friends and relatives.These are preventive measures in order to prevent the development of diseases that greatly affect the health status of the patient and, in the future, may even lead to his death.
Just conduct prevention and treatment of infectious complications.Prophylaxis involves the application of antibiotics.They are not used if the number of granulocytes decreased slightly (1,5h109 / l), and if much reduced (1h109 / l), then use antibacterial drugs.Usually, two broad-spectrum antibiotic (act on different microbes) tablets.If the neutrophils in the blood below 1h109 / l, appointed two antibiotics intravenously or intramuscularly.Typically, a cephalosporin with an aminoglycoside.If infection has already aligned, then apply two or three broad antibiotic spectrum of the same groups (cephalosporins, aminoglycosides, as well as penicillin).When a patient is treated with antibiotics, it is parallel to prescribe antifungal medications (such as Nystatin, levorin and others).The treatment is conducted until the numbers of granulocytes will not appear on the level of the norm.
The treatment also includes a transfusion of leukocyte white blood cells mass or thawed 2-3 times a week.By resorting to this procedure when greatly reduced granulocytes in the blood (do not apply when the immune agranulocytosis).Leukocyte mass is selected to be compatible leukocytes of the patient in the system HLA-antigen.Transfusion of leukocyte mass contraindicated in immune agranulocytosis.It reduces the production of granulocytes in the bone marrow and inhibits the production of antibodies.Applied prednisolone at a dose of 40 to 100 mg per day, to normal numbers of granulocytes in the blood.Then slowly reduce the dose.
required in the treatment of agranulocytosis leykopoeza used stimulants.These include sodium nukleinat, leucogen, pentoxy.Treatment continued for 3-4 weeks.It depends on the condition of the patient.Just apply Molgramostin, Leykomaks.
In severe intoxication use detoxification (fever, pallor, nausea, vomiting, stool disorders, severe weakness, low blood pressure).Apply gemodez, glucose, isotonic sodium chloride solution or Ringer's solution.You can use platelet transfusions in patients with severe hemorrhagic syndrome, which is caused by severe thrombocytopenia.Transfused platelets obtained from one donor.You can also use products-gemostatiki that stop bleeding.These include Dicynonum, aminocaproic acid and so forth. If developed severe anemia, the red blood cell transfusions are used.To prevent complications after transfusion of red blood cells are washed more often used.
patient with agranulocytosis is weakened and "unarmed", as his "weapon" - granulocytes (neutrophils) are produced in small quantities.Agranulocytosis is a serious problem today, which is not yet fully explored by scientists, but many laboratories are working on the prevention and treatment of this disease.
Prevention agranulocytosis is a very important aspect of this syndrome, and so we must always support the immune system with vitamins.In the summer, it is fruit, vegetables, and in winter - a tablet vitamins that can bolster the immune system.