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Leukopenias - Practical hematology of children's age

Table of contents
Practical hematology of children's age
Embryonal hemopoiesis
Morfofunktsionalny characteristic of cells of marrow and peripheral blood
Marrow parenchyma cells
Peripheral blood of children of different age
The system of a hemostasis is normal
Etiology and pathogeny of leukoses
Acute leukoses
Acute leukoses - a preleukosis
Possibilities of a predictive assessment of a course of an acute lymphoblastoid leukosis at children
General principles of treatment of an acute leukosis
Chemotherapeutic drugs
Treatment of an acute lymphoblastoid leukosis
Treatment of myeloid forms of an acute leukosis
Infectious complications and symptomatic therapy of an acute leukosis
Consolidation and maintenance therapy of an acute leukosis
Immunotherapy
Remission and recurrence of an acute leukosis
Inborn leukosis
Neuroleukosis
Myelosis
Lymphogranulomatosis
Gematosarkoma
Macrofollicular lymphoma
Angioimmunoblastny lymphadenopathy
Leukemoid tests
Infectious lymphocytosis
Infectious mononucleosis
Leukemoid tests of different types
Dysfunctions of granulocytes
Leukopenias
Histiocytoses
Histiocytoses - an eosinophilic granuloma
Malignant histiocytosis
Family erythrophagocytal histiocytosis
Accumulation diseases
Nimann's illness — Peak
Angiopathies
Hemorrhagic vasculitis (Shenleyn's illness — Genokh)
Mayokki's purpura
Ataxy teleangiectasia
Entsefalotrigeminalny angiomatosis
Kortiko-meningealny diffusion angiomatosis
Cerebroretinal angiomatosis
Hypertrophic gemangiektaziya
Multiple and huge hemangiomas
Elastic fibrodisplaziya
Coagulopathies
Hereditary coagulopathies
Hemophilia And
Clinic of hemophilia
Treatment of hemophilia
Angiohemophilia
Cristmas disease (Kristmas's illness)
Hereditary deficit of factors of XI, XII, XIII and I
Dysfibrinogenemias
Hereditary deficit of factors of VII, X, V and II
Deficit K-vitaminozavisimykh of factors of coagulation
Syndrome of the disseminated intravascular coagulation
Clinic and diagnosis of the IDCS
Treatment of the IDCS
Thrombocytopenia
Idiopathic Werlhof's disease
Clinic and diagnosis of an idiopathic Werlhof's disease
Treatment of an idiopathic Werlhof's disease
Isoimmune Werlhof's disease
Transimmune Werlhof's disease of newborns
Trombogemolitichesky Werlhof's disease (syndrome Moshkovich)
Hereditary Werlhof's diseases
Trobotsitopatiya
Anemias
The anemias connected with blood loss
Chronic posthemorrhagic anemia
Iron deficiency anemias
Clinic and diagnosis of an iron deficiency anemia
Treatment of iron deficiency anemias
Sideroakhrestichesky, sideroblastny anemias
Megaloblastny anemias
Foliyevodefitsitny anemia
Hereditary forms of megaloblastny anemias
Hereditary dizeritropoetichesky anemias
The anemias connected with oppression of proliferation of cells of marrow
Hereditary hypoplastic anemias
Hemolitic anemias
Hemolitic anemias - an ovalocytosis, a hereditary stomatocytosis
Acanthocytosis, piknotsitoz
The hereditary hemolitic anemias connected with disturbance of activity of enzymes of erythrocytes
The hereditary hemolitic anemias connected with disturbance of structure or synthesis of hemoglobin
The acquired immune hemolitic anemias
Isoimmune hemolitic anemias
Treatment of a hemolitic illness of newborns
Autoimmune hemolitic anemias
List of references

At reduction of number of leukocytes in peripheral blood speak about a leukopenia. In clinical practice this syndrome meets often, however thin mechanisms of development of a leukopenia are not rather studied. At children the level of leukocytes is lower 4,5 X 109/l regard as a leukopenia. Most often reduction of quantity of neutrocytes — a neutropenia meets. States with decrease in other forms of leukocytes are also known: lymphocytopenia, monocytopenia, eosinopenia.
Concept "leukopenia" not a nosological form, but symptom of defeat of white blood. It is big group of diseases with various etiology, a pathogeny and clinic. The general symptom — decrease in quantity of leukocytes. The states which are followed by a neutropenia are most studied. It is diagnosed at absolute quantity of neutrocytes lower than 2 X 109/l. Development of leykopenichesky states can be caused by various pathogenetic mechanisms. Most of authors allocate four main mechanisms, from them main is a decrease in products of neutrocytes. A neutropenia connected with disturbance of products of neutrocytes, are inherent to many morbid conditions of the hereditary and acquired nature. Marrowy disturbances are observed at Kostman's syndromes, Shvakhmana, hereditary hypoplastic anemias, scarce anemias, malignant oncological diseases. They are caused by influence of myelotoxic factors (medicinal substances, chemical agents). Along with reduction of products the slowed-down exit of leukocytes from marrow can be noted. The second mechanism of development of a neutropenia is reduction of quantity of the circulating neutrocytes in a vascular bed as a result of the increased destruction or the increased utilization of leukocytes. Most often destruction of neutrocytes in peripheral blood is connected with immune defeat of cells and is observed at collagenoses, other immune diseases, reception of medicines. The increased destruction of neutrocytes in a spleen (hypersplenism) is noted at various pathology which is followed by a splenomegaly. Neutropenias can be caused by redistribution of neutrocytes in a vascular bed. And, at last, genesis of a neutropenia can have the combined mechanism — disturbance of a marrowy blood formation and the increased destruction of neutrocytes. It is noted at an immune agranulocytosis when there are antibodies against mature neutrocytes circulating in peripheral blood and the antibodies against parent cells breaking marrowy blood formation.
At children allocate two forms of a neutropenia: hereditary and secondary symptomatic. At a neutropenia certain disturbances of phagocytal function of neutrocytes which are inherent to both hereditary, and symptomatic forms are allocated. Phagocytosis undergoes the most expressed changes in the completing phase, at the same time disturbance of a chemotaxis is also noted. Bactericidal properties of neutrocytes decrease as a result of defects in processes of aerobic oxidation and the lowered formation of peroxide of hydrogen. Reduction of phagocytal function of neutrocytes to some extent correlates with weight of process and degree of a neutropenia. Hereditary neutropenias meet rather seldom. The most severe form is the hereditary neutropenia of Kostman — a children's genetic agranulocytosis. The disease is for the first time described by R. Kostman in 1956. In peripheral blood almost completely there are no neutrocytes. The neutropenia pathogeny is completely not studied. The leading mechanism is disturbance marrowy. a hemopoiesis — a delay of maturing of neutrocytes at the level of promyelocytes. At Kostman's illness feature of defect of neutrocytes which consists in lack of maturing of specific granules is revealed. Elektronnomikroskopichesky and cytochemical researches showed that neutrophylic granularity in cells is identified as primary granules; the secondary granularity inherent to mature neutrocytes, it was not succeeded to reveal. At cultivation of marrow the normal growth of cells predecessors, but generally at the expense of monocytic and eosinophilic elements is noted. The part of cells predecessors of a granulocytopoiesis is formed by normal neutrocytes, other part — defective cells. The disease is inherited on autosomal recessively type. The illness is shown already at early children's age, in the period of a neonatality or in the first months of life. At children resilience to infections sharply decreases. The chronic recurrent damages of skin, mucous membranes having a peculiar current are observed. Due to the neutropenia inflammatory processes can not be followed by a gnoyeobrazovaniye, and have ulcer and cicatricial character. The generalized hyperadenosis, frequent pneumonia with formation of cavities are characteristic. Against a neutropenia pulmonary process proceeds also atypically. Radiological it is difficult to establish a stage of consolidation and in most cases state already formulated cavity. Cellular substrate of pulmonary infiltrates is generally provided by plasmocytes. The heaviest displays of an illness of Kostman is generalized septic process. At septic complications increase in a spleen is possible. At children of early age the disease can be suspected on the basis of a recurrent pseuofurunculosis. In peripheral blood at a relative or absolute neutropenia the quantity of leukocytes are normal or is moderately reduced. In a leykogramma neutrocytes or completely are absent, or meet in the minimum quantities (1 — 2%). And inflammatory process develops without blood changes, the neutropenia remains at the former level, being followed by increase in quantity of eosinophilic granulocytes and monocytes of various degree of a maturity. Quite often monocytes have morphological similarity to imperious cells — a loose structure of a kernel with single nukleola. Such picture of blood in combination with a leukopenia, a monocytosis, a generalized lymphadenopathy, ulcer and necrotic defeats can serve as the reason of wrong diagnosis of an acute monoblast leukosis. However at Kostman's illness there are no true blast cells, there are no thrombocytopenia and anemia as platelet and erythrocyte sprouts are kept. At research of marrow find elements of various stages of eosinophilic granulocytes and monocytes in the increased quantity. In sternal punctate, as well as in peripheral blood, mature neutrocytes are absent. The most differentiated form are promyelocytes which percent in a miyelogramma is sharply increased. Promyelocytes contain big azurophilic granularity. The forecast at Kostman's disease adverse. Children perish on the first year of life, is more rare — live several years. A basic reason of death — sepsis and pneumonia. If children nevertheless live up to school age, the illness gets softer current, the frequency of infectious complications decreases. On supervision A. I. Vorobyova (1979), in our country more high-quality option of an illness which is characterized by less heavy course of process is noted. At the same time the differentiation of cells of a neutrophylic row can reach myelocytes and metamyelocytes. Pathogenetic therapy does not exist. Treatment is directed to fight against infectious complications. The main place in it belongs to antibiotics. Scheduled maintenance has important value. In house conditions — this creation of the greatest possible sterility, careful care of the child, processing of skin disinfecting solutions, sanitation of an oral cavity, etc. The positive take at a bone marrow transplantation is probable.
Rather not severe form of a hereditary neutropenia is the family high-quality neutropenia. Pathogenetic mechanisms of a disease are not clear. This syndrome on the kinetic features has similarity to Kostman's syndrome. The main defect consists in disturbance of marrowy maturing of cells of a neutrophylic row. There is a differentiation delay at a stage of myelocytes. The high-quality neutropenia has family character, but unlike Kostman's syndrome is transferred on autosomal dominantly type. The neutropenia is noted at children from early age, however clinical displays of a disease are poor, and in some cases in general are absent. Persistent recurrent infections are rather rare. Most often children have a periodontosis, the general weakness, an adynamy are also characteristic. In certain cases the neutropenia can be shown by nothing and is accidentally diagnosed at research of blood smears. The family high-quality neutropenia can be observed at several members of one family, it is characterized by a constant leukopenia within 2X 109/l — ZH 109/l. In peripheral blood the absolute and relative quantity of neutrocytes is reduced. In a leykogramma contents makes them 20 — 25%. In red blood and thrombocytes there are no changes. In a miyelogramma the quantity of cells are normal, the number of forms of a neutrophylic row — metamyelocytes, band, segmentoyaderny leukocytes is reduced. The syndrome is characterized by a high-quality current, children live up to adult age. Special therapy is not required. Infectious diseases treat by the general principles.
The periodic (cyclic) neutropenia at which of peripheral blood neutrocytes regularly disappear belongs to rare syndromes. The period of a neutropenia develops through intervals of 2 — 4 weeks and 4 — 5 days proceed. Duration of light intervals is individual at each patient and is, as a rule, constant. During intervals hematologic indicators are normalized. The pathogeny of a disease is not clear. Suggest about the disturbance of marrowy maturing of neutrocytes caused by defect of regulators of a haemo cytopoiesis. One of the possible reasons is deficiency of haemocytes at the level of parent stem cells as along with defeat of a granulotsitarny sprout cyclic changes of platelet and erythrocyte sprouts can be observed. The disease has hereditary character. And cases both with autosomal and recessive, and with autosomal and dominant type of transfer are described.
Clinical symptoms observe from early children's age. However T. S. Istamanova and V. A. Almazov (1961) who in detail described this pathology in our country note that at some patients the cyclic neutropenia can be shown at advanced age. However, authors point that neutropenias of children's age and elderly persons, perhaps, have various pathogenetic mechanisms. At some persons the disease can proceed without clinical manifestations. The period of a neutropenia is followed by an aggravation of symptoms of patients — the general weakness, slackness, fatigue develop. The mialgiya, an arthralgia, temperature increase are noted. In the period of hematologic crisis nonspecific protection considerably decreases and the organism becomes vulnerable for different infections. Most often there are quinsy, damage of upper airways, pneumonia with abscessing. Pustulous damages of skin — abscesses, furuncles, ulcer and necrotic damages of mucous membranes of an oral cavity, stomatitis, conjunctivitis develop. The hyperadenosis, a liver, a spleen meets seldom. During the acute period in peripheral blood reveal a leukopenia, neutrocytes completely are absent. In a leykogramma — a monocytosis and an eosinophilia. The cyclic neutropenia can proceed also without decrease in leukocytes owing to compensatory increase in number of monocytes and eosinophilic granulocytes. Sometimes neutropenias accompany anemia and thrombocytopenia. Anemia can also form because of infectious process. In a miyelogramma there are no forms of neutrocytes, the following behind a stage of promyelocytes, the number of the last and myeloblasts is increased.
Also the maintenance of monocytes and eosinophilic granulocytes increases. The hematologic picture keeps 3 — 5 days, then there is a spontaneous recovery of composition of peripheral blood. At simultaneous research of marrow and peripheral blood of full identity of changes is not present. The picture of a miyelogramma advances indicators of peripheral blood. At a neutropenia in marrow there is already a recovery of a normal haemo cytopoiesis. Or on the contrary: mature neutrocytes already in it disappear, and in peripheral blood still everything is safe. During the light period indicators of a gemogramma and a miyelogramma do not differ from norm, then again the maintenance of granulocytes sharply decreases.
Forecast of a disease rather favorable. Children with a cyclic neutropenia have the heaviest infectious processes causing a possible lethal outcome, are noted at early age. Than the child is more senior, that the forecast is more favorable as over time the disease proceeds more softly, and infectious complications — are less frequent and heavy. A number of authors indicate certain difficulties of diagnosis of a cyclic neutropenia. It is caused by the fact that during the light period there are no changes. Usually infectious process is the cause for inspection of the child, however at the time of a blood analysis of change of a gemogramma can already pass. Perhaps, the disease rarity connected with difficulties of diagnosis is explained by it. Therapy is generally directed to fight against infectious complications. Apply antibiotics of a broad spectrum of activity, gamma-globulin. With the established cycle duration for the purpose of prevention it is reasonable to patient to appoint antibiotics some days before hematologic crisis. For stimulation of a haemo cytopoiesis and prevention of crisis at one time used hormones — glucocorticoids, androgens, however the desirable result is not received. In literature there are separate messages on improvement after a splenekomiya. However surgical intervention is not shown to patients as it has no pathogenetic justification and unconditional efficiency. The balanced diet, the mode, fortifying procedures are important for normal development of the child.
In 1952 Vahlquist, N. Anjou and S. Gasser described the chronic granulocytopenia of children's age known still as Valkvist's syndrome — Gassera. The disease is characterized by lack of segmentoyaderny neutrocytes in peripheral blood. Were suggested that it is caused by the increased destruction of granulocytes in a blood channel. However in marrow the delay of maturing of neutrocytes at a stage of band forms is also noted that testifies to defects of a haemo cytopoiesis. The disease arises at children of early age and is shown by the recurrent infections having rather easy current. Children lag behind in physical development, temperature increase is periodically noted. Recurrent diseases of upper respiratory tracts are most often observed. Such pathology as purulent damages of skin, ulcer and necrotic damages of mucous membranes, pneumonia, usually do not complicate the course of a disease. The lymphadenopathy, insignificant increase in a liver and spleen are sometimes noted. In peripheral blood the quantity of leukocytes, erythrocytes and thrombocytes, and also level of hemoglobin meets standard. In a leykogramma there are no segmentoyaderny neutrocytes in the presence of metamyelocytes and band forms, there is a monocytosis. The fact that on inflammatory processes the neutrophylic reaction though inadequate to an irritant is noted is interesting. There can be segmentoyaderny granulocytes, but the quantity of metamyelocytes and band generally increases. In marrow cellular ratios are kept, but there is a break of maturing of neutrocytes at a stage of band leukocytes. The disease is characterized by a chronic high-quality current, without life-threatening infectious processes. After a row of years, usually at preschool age, most of patients has a spontaneous recovery without the subsequent return of clinical and hematologic symptoms. Family character of a disease is not established. A part is played by a premorbidal background. Valkvist's syndrome — Gassera is more often noted at children with anomalies of the constitution. Treatment of infectious complications is carried out.
In 1964 N. Schwachman and coauthors described the neutropenia which was followed kistofibrozy a pancreas. The mechanism of development of a syndrome of Shvakhman is not clear as damage of a pancreas cannot exert impacts on a haemo cytopoiesis. Deficiency of stem cells is the cornerstone of a granulocytopenia. Along with a neutropenia thrombocytopenia and anemia can develop. The disease is clinically shown at early age and characterized by signs of defeat of two systems — hemopoietic and digestive. The frequent liquid fetid chair, a steatorrhea is noted. Children badly gather in weight, gipotrofichna. At many patients disturbance of development of an epiphysis and forming of orthopedic pathology is observed. At some children the galactosemia is possible that it leads to development of a gepatosplenomegaliya, defeat of a nervous system — to lag in psychomotor development. The raised susceptibility to infections is noted. In peripheral blood — a neutropenia of various degree, up to total absence of segmentoyaderny forms. The quantity of erythrocytes and thrombocytes is reduced. Anemia of generally hypochromia type, iron deficiency, caused by disturbance of absorption as a result of a kistofibroz of a pancreas. At infectious process neutrophylic reaction, but smaller intensity, than normal is observed. In marrow the picture is very various: from a moderate hypoplasia to a moderate hyperplasia with a delay of maturing of cells of a neutrophylic row at a metamyelocyte stage. Results of research of enzymatic activity demonstrate sharp reduction of content of proteolytic enzymes or their total absence. In some cases the specific clinical picture of a syndrome of Shvakhman can be the cause of wrong diagnosis of a mucoviscidosis. The convincing test is the normal level of chlorides. The disease has hereditary character, in literature family cases are described. The forecast improves patients, the most adverse with age — at chest age when consecutive infections can, lead to a lethal outcome. The patient carries out the complex treatment including replacement therapy of a kistofibroz of lodzheludochny gland, an antibioticotherapia of infectious complications, correction of hypochromia anemia ferriferous drugs. For the purpose of stimulation of a haemo cytopoiesis purpose of corticosteroids and androgens is possible.
Rare inborn pathology is Tsinsser's syndrome — Cole — Engmena, F. Zinnsser described in 1910 and N added in 1926. N of Cole and M. of Engman. The syndrome represents a dyskeratosis of skin and mucous membranes in combination with hematologic changes — anemia, thrombocytopenia, a neutropenia. The pathogeny of a disease is not clear. Suggest about internal defect of trunk haemocytes, disturbance of hemopoietic microenvironment, immune mechanisms. The case of a family dyskeratosis at twin brothers is described, however the question of hereditary character of a syndrome is open. Clinical symptoms appear rather late — at youthful age and at adults. Skin and its derivatives, mucous membranes are surprised. Multiple scattered hyperkeratosis with preferential localization on a face, a neck, a back, a breast, an atrophy of skin of palms and a foot, dystrophy of nails, a hypotrichosis of eyelashes, damage of mucous membranes of an oral cavity, generally language and gums are observed. Function of closed glands is broken — the nanism, an underdevelopment of secondary sexual characteristics is characteristic. The picture of peripheral blood is very various. Anemia, thrombocytopenia, a neutropenia can be shown as separately, and the general pancytopenia. At Tsinsser's syndrome — Cole — Engmen in marrow there is no uniform picture: states from a gipotsellyulyarnost to a gipertsellyulyarnost are noted. Due to the development of a pancytopenia the forecast of a disease adverse. A symptomatic treatment, there is no effective specific therapy, use of corticosteroids and androgens is possible.
The syndrome of a neutropenia is quite often observed at primary hereditary immunodeficiency at which there are defects in T - and V-lymphoid systems. Considering a significant role of these systems in regulation of a granulocytopoiesis, there is clear a development of a neutropenia at primary immunoparesis.
The general clinical signs of all immunodeficiency are the persistent recurrent infections caused as decrease in a specific immune responsiveness, and nonspecific protective forces (phagocytosis). The granulocytopenia is quite often observed at an agammaglobulinemia (Bruton's illness) — a syndrome at which there is an inborn disturbance of synthesis of all fractions of immunoglobulins. It is characterized by development of heavy it is purulent - septic complications. It is inherited on the recessive type linked to a floor. The neutropenia of heavy degree is observed at the combined Swiss type of immunoparesis. It is inherited on the recessive type linked to a floor. In a clinical picture the basic is the septic process leading to a lethal outcome. The inborn hypoplasia or an atrophy of a thymus is also characteristic of a disease.
Treatment of hereditary immunodeficiency is carried out in two directions: 1) active antibiotiko symptomatic therapy of infectious complications; 2) the specific replaceable and stimulating therapy. Apply high doses of gamma-globulin, a blood plasma transfusion to these purposes. Now it is proved that antivermicular drug levamisole (decarice) is an immunomodulator of T-system of lymphocytes. Preventive actions — keeping of the child in the greatest possible conditions of an asepsis have important value. In literature cases of effective treatment by means of a bone marrow transplantation are described.
Immune mechanisms cause development of the symptom complex described in 1958 by M. Stefanini as a passing neutropenia of newborns. Now allocate two possible options of development of a neutropenia of newborns. The first is close on the pathogeny to a hemolitic illness of newborns and results from development in an organism of mother of the antibodies directed against neutrocytes of the child. Anti-neutrophylic antibodies get through a placenta and, causing destruction of leukocytes, lead to development of a neutropenia — an isoimmune way of transfer. The second possible mechanism is transplacental transfer of antibodies from mother suffering from a neutropenia, to a fruit. Afterwards there is a development of antibodies already in the organism of the child — the autoimmune mechanism. The neutropenia of varying severity is observed at children at once after the birth. At the same time terrible infectious complication joins very seldom. Hematologic changes have passing character, in 1 — 2 month after the birth the composition of peripheral blood is normalized. At disease height in marrow products of neutrocytes are lowered. Forecast of a passing neutropenia of newborns favorable. An antibioticotherapia is effective at treatment of secondary complications.
Rather high-quality current characterizes a syndrome of "lazy" leukocytes. At patients disturbance of elimination of neutrocytes from marrow in peripheral blood at a normal haemo cytopoiesis is noted. It is established that physical activity of granulocytes — as directed (chemotaxis), and accidental is broken. Now it is connected with deficiency of cellular membranes. Other stages of phagocytosis and bactericidal properties of cells do not suffer. The neutrocytes which are found in peripheral blood possess bigger motive ability, than marrowy cells as in a blood channel the most active granulocytes break. The clinical syndrome of "lazy" leukocytes is shown by frequent infectious diseases. Damage of upper airways is on the first place. At children long subfebrile condition, otitis, stomatitis are noted. In peripheral blood — the considerable neutropenia, reactions to infectious processes is not noted. In marrow the quantity of mature gianulotsit is increased at safe relationship of sprouts of a haemo cytopoiesis. The forecast at this syndrome rather favorable as heavy infectious complications are noted seldom. Therapy of secondary inflammatory diseases is carried out. The specific therapy stimulating a physical activity of neutrocytes is not developed.
Leukopenias often have secondary, symptomatic character. Development of a neutropenia is promoted by various etiological factors and pathogenetic mechanisms. Falling of number of leukocytes and granulocytes is noted at reception of some medicines. At the same time in a pathogeny two mechanisms matter. The granulocytopenia can be a consequence of direct toxic effect of medicinal substances on a haemo cytopoiesis. It is peculiar to all cytostatic drugs. Ionizing radiation has the same effect. The second mechanism is the increased destruction of neutrocytes in a peripheral bed — medicamentous gaptenovy leukopenias. Medicinal substances (haptens) when fixing on granulocytes lead to change of their antigenic structure and formation of antigen, "alien" for an organism. Response are products of antibodies that leads to destruction of leukocytes in the course of immunological reaction. To number especially dangerous, in the development plan for a leukopenia, drugs pyrazolon derivatives (pyramidon, analginum, Butadionum), streptocides, the majority of antibiotics known now belong. And use of these drugs is fraught not only development of a moderate neutropenia, but also such terrible syndrome as an acute agranulocytosis. The listed medicinal substances very widely apply in pediatric practice and is quite frequent without due hematologic control. Therefore (1 time in 5 — 7 days) research of peripheral blood has to be a necessary condition regular. At cytostatic therapy of tumoral diseases drugs cancel on the basis of an individual assessment of a haemo cytopoiesis and general provisions of therapy of this pathology. With the replaceable purpose apply transfusions of components of blood (leykomassa).
The big group is made by the immune neutropenias caused by the increased destruction of neutrocytes in peripheral blood. Etiological factors of this form are viral infections, bacterial toxins. The symptomatic immune chronic neutropenia is observed at the tumoral diseases, collagenoses, a gromerulonefrita and many other states which are followed by autoimmune aggression.
The symptomatic leukopenia with a neutropenia can be observed at the diseases which are followed by a splenomegaly. The mechanism of development of a neutropenia at a splenomegaly difficult. In these situations speak about the so-called phenomena of a hypersplenism. There are two points of view on a leukopenia pathogeny at a splenomegaly. On the one hand, the spleen can exert the braking impact on a haemo cytopoiesis that leads to disturbance of maturing of marrowy elements. On the other hand, the spleen is "cemetery" of uniform elements of blood and at a splenomegaly in it the increased destruction of leukocytes exceeding education processes is observed. The leukopenia can be observed at accumulation diseases (an illness to Gosha, Nimann's illness — Peak), cirrhosis with involvement in process of a spleen, a pseudorheumatism (Felti's syndrome) and others.
At identification of a leykopenichesky syndrome the careful analysis is necessary for identification of the reasons and the leading pathogenetic mechanisms as it in many respects defines rational therapy.
Reticular dysgenesis, or inborn lack of leukocytes. Total absence of all forms of leukocytes is noted. The pathogeny of a disease is not clear. Clinical symptoms appear in the period of a neonatality and are characterized by the heavy infection leading to a lethal outcome within several days. In peripheral blood leukocytes are absent at normal quantity of erythrocytes and thrombocytes. In marrow the myeloid sprout of a hemopoiesis is reduced, erythroidal and platelet sprouts are not broken. At research of a thymus gland, lymph nodes, spleens lack of lymphocytes is noted. An antibioticotherapia is inefficient.
The most severe form of a leukopenia is the acute agranulocytosis. It is a hematologic syndrome at which reduction of quantity of leukocytes lower than 1 X 109/l or neutrocytes lower than 0,75 X 109/l is noted. Etiological factors of an agranulocytosis are various. It can develop at reception of sulfanamide drugs, pyramidon, analginum, acetylsalicylic acid, acetphenetidiene, Butadionum, barbiturates, Tubazidum, Ftivazidum, cytostatic means.
Salts of heavy metals, toxic substances (benzene and its derivatives, insecticides), infections can be an etiological factor. Cases of an acute agranulocytosis at levamisole reception are described. Allocate two types of an agranulocytosis depending on the leading pathogenetic mechanism.
The myelotoxic agranulocytosis develops as a result of impact on marrow of some factors oppressing a haemo cytopoiesis. In particular, it is noted at treatment by cytostatic drugs.
The immune agranulocytosis arises owing to the increased destruction of leukocytes in a peripheral bed. The immune agranulocytosis is divided on gaptenovy and autoimmune. The majority of medicamentous options of an agranulocytosis is gaptenovy. The autoimmune agranulocytosis develops at various autoimmune processes, collagenoses, a glomerulonephritis etc. In emergence of an acute agranulocytosis both immune, and myelotoxic mechanisms can operate. In certain cases the etiology and a pathogeny are not clear and then speak
about genuinical forms of an agranulocytosis. Probably the hereditary defects of leukocytes which are shown on a certain adverse background can be the cause of their emergence.
The clinical picture of a myelotoxic and immune agranulocytosis has similar lines. Often emergence of typical symptoms of an illness is preceded by the eclipse period with febricula symptoms, a headache, appetite loss. At the height of a disease sharp weakness, pallor, fever are observed (is more often than remittiruyushchy type). In clinical symptomatology of an agranulocytosis ulcer and necrotic defeats of an oral cavity, necrotic quinsies are noted. The pharyngalgia disturbs, at survey on almonds the dirty-gray plaque reminding diftericheskiya is found then necrotic ulcers and plaques are formed. Except almonds, the nekrotization takes palatal handles, a uvula, gums, a soft and hard palate. Frequent infectious complication is the pneumonia causing destruction of pulmonary fabric. The digestive tract — a necrotic enteropathy can be involved in process. It is characterized by a frequent liquid chair, an abdominal pain, fever, high intoxication. Necroses at the same time can be localized in peyerovy plaques of a small bowel, a gullet, a large intestine. Terrible complication of an agranulocytosis is the septic process which is followed by forming of the multiple metastatic centers. Weight of a clinical picture depends on degree and duration of an agranulocytosis. Most hard the forms which are characterized by a total disappearance of granulocytes that, for example, is observed at a gaptenovy medicamentous agranulocytosis proceed. If there is a recovery of a granulocytopoiesis, then it substantially facilitates the course of infectious process and promotes an absolute recovery. On supervision of A. E. Baranov and coauthors (1981), at the level of nentrofilny granulocytes within 0,5Õ109/l heavy infectious processes do not develop, and those that arose, as a rule, easily are stopped.
The blood picture at all forms of a disease is characterized by decrease in quantity of leukocytes and neutrocytes, up to a total disappearance. In the remained neutrocytes find degenerative changes of a kernel, in cytoplasm toxic granularity is noted. Motor and their phagocytal performance is reduced. Sometimes at height of an agranulocytosis there is a monocytic reaction, quite often in blood plasmocytes meet. At a myelotoxic agranulocytosis observe the anemia and thrombocytopenia which is followed by a hemorrhagic syndrome. The picture of marrow depends on a type of an agranulocytosis. At a myelotoxic agranulocytosis find a marrow devastation, a reduction of all sprouts of a hemopoiesis. At immune forms of an agranulocytosis in marrowy Tsunktat reduction of quantity miyelo-and metamyelocytes and mature forms of neutrocytes is noted. Eritro-and megakariotsitarny sprouts usually do not suffer.
Treatment of both forms of an agranulocytosis is directed to fight against infectious complications. Therapy has to be complex. In detail basic principles of therapy are stated in the section "Infectious Complications and Symptomatic Therapy of Acute Leukoses".
Let's note the main moments: 1. Creation of conditions of the greatest possible sterility. 2. Use of antibiotics of a broad spectrum of activity. 3. Replacement therapy by blood components. 4. Rehabilitation and symptomatic therapy (disintoxication, vitamin therapy, maintenance of cardiovascular activity).
Now it is proved that use of glucocorticoids at an agranulocytosis is inexpedient, even in the presence of immune forms. They have no pathogenetic effect in already developed acute phase of an agranulocytosis and, besides, reduce anti-infectious immunity (A. I. Vorobyov and soavt., 1979), aggravating the course of infectious processes (V. A. Almazov and soavt., 1981). As the indication to purpose of corticosteroid hormones recurrence of an immune agranulocytosis can serve (V. A. Almazov and soavt., 1981). Replacement therapy by blood components (leukocytic weight) is recommended to carry out at hard prolonged cases.
At gaptenovy immune forms of an agranulocytosis timely drug withdrawal, caused reaction is important, afterwards reception of this medicine is inadmissible.



 
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