Leukopenia - is a reduced level of white blood cells in the total blood cell composition to the border less than 1,5 × 109 / L.In case there is a total absence of blood in the blood, develop a condition called "agranulocytosis".The incidence of leucopenia with severe amounts to less than 1 case per 100 000 population, and the incidence of congenital form of leukopenia is 1 in 300,000 people.Mortality from leukopenia of varying severity is within 4-40%.
The structure prevail incidence of leucopenia provoked by the action of chemotherapeutic drugs used in cancer.
Besides funds for chemotherapy, adverse reactions in the form of leukopenia has a large number of different groups of drugs (neuroleptics, hormones), so the dosage form of leukopenia over half of the population suffers elderly.For women is more common occurrence of this disease than men.
polyetiology Leukopenia is a syndrome that can act both as a primary condition, and is a complication of other
- a congenital defect in the genetic field that is transmitted in an autosomal recessive manner, so the innate leukopenia can be attributed to the group of sporadic diseases;
- myelotoxic leucopenia provoked by the action of chemotherapeutic drugs used not only in hematologic malignancies, but also for systemic oncological processes in the body;
- direct lesions of bone marrow, which is the central body of hematopoiesis;
- violation of the metabolic processes in the body, accompanied by a lack of B vitamins, copper and folic acid;
- infection of the body (cytomegalovirus, rubella virus, Epstein - Barr virus, hepatitis, AIDS);
- different forms of tuberculosis;
- medicines used as therapy of diseases of various organ systems (mercury diuretics, nonsteroidal anti-inflammatory drugs, antidepressants, antihistamines, and anti-thyroid);
- rheumatoid arthritis and kidney failure, which is used for the treatment of captopril and probenecid, often provoke signs of leukopenia;
- long work with chemicals (benzene, pesticides).
There are three main etiopathogenetic mechanism of leukopenia: violation of the production of white blood cells in the blood, circulating leukocytes failure or redistribution, as well as accelerate the destruction of neutrophils.
Normally, white blood cells are located in bone marrow, circulating blood are transported to all vital organs and tissues.In the blood, there are two types of leukocytes: freely circulate through the bloodstream and adherent to the vessel wall.The average length of stay of leukocytes in peripheral blood is less than 8 hours, after which they are redistributed into tissue.Excessive destruction of the white blood cells can be triggered by exposure to antileykotsitarnyh antibodies.
Under the influence of chemotherapy and radiation exposure in the bone marrow is a violation of the first level, that is, mature white blood cells are not formed due to the mass death of young proliferating cells in the bone marrow.
If infectious lesions and bacterial sepsis is a sharp decrease in circulating free fraction by increased leukocyte adhesion to the vascular wall, provoked by the action of endotoxins.Any parasitic defeat of the body takes place with splenomegaly and excessive accumulation of white blood cells in the spleen, and therefore, there is an insufficient level of white blood cells in the blood.
Congenital forms of leukemia, as well as leukemia or aplastic anemia, characterized by the violation of the main bone marrow stem cells, from which the proliferation of white blood cells.
for HIV infection and AIDS is characterized by a destructive effect on bone marrow stromal cells, which leads to a failure in the hematopoietic system, as well as accelerated degradation of existing mature leukocytes, found in the blood.
Chronic administration of certain groups of drugs, leukopenia is due to toxicity, immune and allergic effects on the body as a whole and in the central organs of hematopoiesis in particular.
Typically, radiation does not manifest itself, that is, there is no specific clinical symptoms, show a decrease in white blood cell count and are not characteristic of other diseases.
slight decrease in white blood cells can cause absolutely no complaints on the part of the patient's symptoms and health problems occur when joining or bacterial infectious complications.Also, a large value has a duration of flow of leukopenia.Thus, if leukopenia at 0, 1 × 109 / L observed for seven days, the risk of infectious complications is less than 25%, while the same duration of leukopenia 1.5 months in 100% of cases accompanied by bacterial or infectiouscomplications of varying severity.
on the risk of infectious complications important factor is the speed and dynamics of the development of leukopenia.There is a definite relationship - the faster decreases the level of white blood cells circulating in the blood, the higher the risk of infectious complications in the patient, and on the other hand, people with leukopenia indolent less prone to complications.
most important and most often the primary sign of the emergence of infectious diseases on the background of leukopenia is increased body temperature.It should be noted that individuals who are treated with hormonal drugs are not suffering a fever, even when joining the infectious agent.In 50% of cases of febrile patients with leukopenia can not reliably determine the cause and source of infection, as in most cases the focus of infection is endogenous flora, long located in the inactive state, and forming colonization infectious foci.
main focus of infection which is actively populated by pathogenic microorganisms in leukopenia, is the oral cavity.Therefore, along with fever, patients suffering from leukopenia, often complain of painful ulceration on the mucosa of the oral cavity, gingival bleeding, pain during swallowing and hoarseness.
Separately should consider clinical peculiarities of leukopenia as a manifestation of cytostatic disease resulting from exposure to drugs that are used as chemotherapy for cancer.This disease is characterized by loss of all germs of blood, and therefore, there is a decrease the level of red blood cells, white blood cells and platelets, which manifests itself not only in a fever, and hemorrhagic syndrome (bleeding and hemorrhage), of anemia (weakness, pallor) syndromenecrotic enteropathy (abdominal pain without clear localization, loose stools, flatulence) and oral syndrome (necrotic ulcerative stomatitis).
Unfortunately, cytostatic disease, one manifestation of which is leukopenia, often complicated by the addition of not only the infection, but bacterial septic lesions of the body, often ending in death.In bacterial septic defeat marked the rapid growth of clinical symptoms until the appearance of signs of septic shock (acute cardiovascular and respiratory failure).
If a patient shows signs of leukopenia, the inflammation occurs with some features.For example, inflammation of the soft tissues are mild local displays (slight pain and redness of the skin), but the overall intoxication syndrome sufficiently expressed.
special feature of bacterial pneumonia, occurred against the backdrop of leukopenia, is the absence of radiological signs in patients with severe intoxication syndrome.
Patients with leukopenia frequent complication is necrotizing enterocolitis, manifesting minor intestinal manifestations, but the rapid development of peritonitis and perianal inflammation (abscess).
Against leukopenia in 10% of cases, symptoms mioklostridialnogo necrosis - acute muscle pain, swelling, and an increase in the volume of soft tissue, the presence of intramuscular emphysema on X-rays.Mioklostridialny necrosis is rapid during spontaneous and quickly complicated by septic shock.
frequent manifestations of reducing the number of white blood cells in the female body is leukopenia uterus, which manifests itself changes of the menstrual cycle in the form of increased menstrual flow, irregular menstruation, and uterine bleeding.
To assess the severity of disease in a patient there is a generally accepted international grading leukopenia.According to the classification of the severity of leukopenia release third degree.
When I degree leukopenia observed white blood cell count of up to 1,5 × 109 / L, and at this stage there is a minimal risk of bacterial complications in the patient.
for II degree of leukopenia characterized by reduction of circulating white blood cells to the level of 0,5-1,0 × 109 / L.The risk of infectious complications attachment is not less than 50%.
III degree of leukopenia, which has the second name "agranulocytosis" characterized by a sudden lack of white blood cell count in the blood up to the border of less than 0,5 × 109 / L, and is often accompanied by infectious complications.
addition leukopenia division of severity, there is a classification of the state, depending on the length of the existence of its features.For the duration of leukopenia divided into acute (duration changes in the laboratory does not exceed three months) and chronic (long-term course of the disease, more than three months).
acute leucopenia should be considered as a transient state, observed in various viral diseases.Chronic leukopenia has more sophisticated mechanisms of formation and is divided into three forms: autoimmune, congenital or idiopathic (cause leukopenia can not figure out), redistributive (transfusion and anaphylactic shock types, in which the observed accumulation of white blood cells in the tissues of the intestines, lungs and liver).
for children ages criterion establishing leukopenia is the reduction of leukocytes of less than 4,5 × 109 / L.As a rule, we are talking about reducing the number of leukocytes in children, is meant reduced level of neutrophils.
most common cause of neutropenia in children are infectious diseases - namely, mumps, measles, rubella, influenza and tuberculosis.The presence of leukopenia in the septic state is a poor prognostic sign.
In childhood, leukopenia, often acts as a side reaction to the reception of various drugs, which include: antibiotics (chloromycetin, streptomycin), antihistamines, sulfonamides and organic arsenic compounds.
leukopenia in the neonatal period is a component of pathological conditions such as megaloblastic anemia, pancytopenia and aplastic anemia Fanconi.Children with congenital gamma globulinemiey, subject to the emergence of recurrent forms of granulocytopenia.
Feature leukopenia in children is the rapid development of clinical manifestations and the rapid accession of infectious complications.For childhood little sluggish characteristic form of leukopenia, which greatly facilitates the early diagnosis of the disease state.
Due to the fact that childhood is very rare isolated leukopenia, more attention should be given to diagnostic activities, contributing to the establishment of the correct diagnosis.So, in addition to the standard count of white blood cells in the total blood analysis and consideration of leukocyte, children with severe leukopenia, combined with anemia and thrombocytopenia shown holding additional methods of research:
- a blood test for the presence of immature blast cells;
- puncture or biopsy of the bone marrow in order to determine the pathogenesis of leucopenia (leykopoeza glitch in the system, accelerating the destruction of cells in the peripheral blood and the presence of blast cells);
- blood tests for the presence of antinuklernyh antibodies, rheumatoid factor, antibodies antigranulotsitarnyh;
- biochemical blood tests, liver function tests, markers of viral hepatitis, levels of B vitamins
We should also mention the transient neonatal form of leukopenia, which is a variant of the norm and does not require medical correction.
Transient leucopenia occurs in breast period due to the impact of child and maternal antibodies that reach the body of a child in utero.In this condition, an infant is observed sustained reduction in the level of granulocytes (within 15%), with preservation of normal values the total number of leukocytes.Typically, this condition alone is held when the child reaches the age of four.
There is a hereditary form of congenital leukopenia, which is a separate disease entities - "disease Kostmana."The first case of the disease was registered in 1956, and at the same time has been clarified the nature of its origin.It turned out that it is hereditary leukopenia family type is transmitted in an autosomal recessive manner and is manifested in the form of a complete lack of neutrophilic granulocytes in the circulating blood.Children born with the syndrome at birth Kostmana subject to severe complications in the form of infectious diseases, since they do not own immunity (abrasions, periodontal disease, abscessed pneumonia, hepatitis absediruyuschy).The main pathogenetic justified drugs used to treat children with the syndrome Kostmana, a colony stimulating factor.
for effective treatment of patients with severe leukopenia should be isolated in a separate room, which will have access only to the medical staff, to use all protective equipment (gown, medical cover, shoe covers, gauze bandage and processing of hand antiseptic).According to the recommendations
hematologists, non-severe form of leucopenia, patients do not require specific treatment and all medical activities should be focused on prevention of possible infectious complications, as well as the elimination of the root causes of this disease.
Severe leukopenia requires an individual and comprehensive approach to the treatment and includes such directions:
- etiopathogenetic treatment, ie, treatment of the underlying disease, provoked leucopenia;
- maintenance of bed rest in a sterile environment;
- correction of feeding behavior;
- preventive measures to prevent possible infection;
- hormone replacement therapy;
- exchange transfusion of leukocyte mass (in the absence of antibodies to leukocyte antigens);
- stimulation of leucopoiesis.
large role in the treatment of leukopenia plays etiological focus of therapy, that is, if there is an autoimmune agranulocytosis or aplastic anemia, it is advisable in this case, the use of immunosuppressive therapy (azathioprine daily dose of 1 mg per 1 kg of weight, methotrexate 15 mg per day rate of not less than5 days at a daily dose of cyclosporine 10 mg per 1 kg body weight).per day.