Hemorrhagic diathesis in children: symptoms, diagnosis and treatment. Differential diagnosis of hemorrhagic diathesis Hemorrhagic diathesis Pediatrics

Hemorrhagic diathesis is a name for a number of diseases that share one leading feature - the body's tendency to spontaneous bleeding. This is a fairly common group of diseases that occur at any age, including early childhood. Hemorrhagic diathesis are congenital, resulting from inherited genetic abnormalities, and acquired, resulting from diseases of the blood or blood vessels.

Since blood coagulation is based on the mechanism of platelet aggregation (gluing), such conditions when bleeding is caused by a violation of this mechanism are called disaggregation thrombocytopathy. Disaggregation thrombocytopathy is the most common direct cause of hemorrhagic diathesis. In second place are violations of the permeability of the vascular wall.

Hemorrhagic diathesis in children

Primary, or acting as an independent disease, hemorrhagic diathesis in children usually has causes either of a hereditary nature: hemophilia, Osler-Randu disease, von Willebrand disease, etc., or immune: Sheinlein-Genoch disease or hemorrhagic vasculitis, of various kinds erythema.

Hemorrhagic diathesis in children can also be a secondary condition, a symptom of a malignant blood disease such as acute lymphocytic, myeloma, or leukocytic leukemia.

In a separate group, functional thrombocytopathies in children are taken out, which are not a disease, but only a manifestation of age-related immaturity of platelets. Functional thrombocytopathies in children are very common, according to statistics, from 5 to 10% of all children are affected by them, and more than 50% of all cases of spontaneous bleeding in children are explained by this condition. Functional thrombocytopathy in children is transient - as a rule, after the completion of puberty, they disappear. However, they cannot be treated lightly, since, when attached to some pathogenic factors, they can play the role of a trigger for life-threatening conditions, for example, cause internal bleeding with a bruise or even a stroke. Age-related thrombocytopathy manifests itself in the same way as other types of hemorrhagic diathesis in children, with increased bleeding, and therefore all such cases require attention. It is impossible to distinguish functional thrombocytopathy from, say, manifestations of acute leukemia at an early stage by external signs; this can be done only after laboratory blood tests.

Types of hemorrhagic diathesis, depending on the causes that caused them

Depending on what mechanism underlies the increased bleeding, 4 groups of hemorrhagic diathesis are distinguished:

• Hemorrhages, which are based on disorders associated with platelets, blood clotting cells: a decrease in their number (thrombocytopenia) or a violation of their function (thrombocytopathy). They are often caused by impaired immune mechanisms, diseases of the liver and kidneys. This group includes all cases of disaggregation thrombocytopathy, including the functional one described above;
• Hemorrhagic diathesis, which arose due to a violation of the metabolism of fibrin, a protein responsible for blood coagulation. Such diathesis can occur under the influence of fibrinolytics, i.e. drugs that reduce the content of fibrin in the blood are also hereditary (hemophilia);
• Diathesis, which are based on both causes, both clotting disorders and disorders of platelet hemostasis. These include hemorrhages at elevated doses of radiation, with malignant blood diseases, von Willebrand's disease;
• Hemorrhagic diathesis, the formation of which is caused by violations of the vascular wall, as a result of which it becomes permeable to blood cells. This group includes hemorrhagic vasculitis, vitamin C deficiency, the consequences of a viral infection.

Symptoms of hemorrhagic diathesis

The main and often the only symptom of hemorrhagic diathesis is increased bleeding, all other signs are somehow connected with it. Increased bleeding manifests itself in the form of prolonged or heavy bleeding for inappropriate reasons, for example, a small scratch can cause serious and prolonged bleeding. Often bleeding is generally spontaneous. This manifests itself in the form of bleeding from the nose, gums, uterine bleeding in women without a cause, the sudden appearance of hematomas (bruises) without previous trauma.

There are 5 types of bleeding in hemorrhagic diathesis:

• Capillary bleeding, manifested on the skin and mucous membranes in the form of a scattering of small red dots (petechiae, ecchymosis), as well as in the form of "oozing" bleeding - nasal, gingival, uterine, gastric, intestinal. They are characteristic of thrombocytopenia and disaggregation thrombocytopathy;
• Hematoma bleeding - the formation of subcutaneous hematomas and internal hemorrhages. Characteristic of hemophilia and some other conditions;
• Mixed type, combining the signs of both capillary and hematoma bleeding, characteristic of hemoblastoses (leukemia, lymphocytic leukemia, etc.);
• Purple bleeding is a small spotted rash that appears symmetrically at first on the legs, then spreads higher to the thighs and buttocks. As the disease progresses, the rash increases in size and may coalesce into large patches. Above the waist, it is rarely formed, although this is not excluded. Such external manifestations of bleeding are a characteristic sign of hemorrhagic vasculitis (Scheinlein-Genoch disease);
• Microangiomatous bleeding, which is based on a hereditary pathology of small blood vessels. Manifested in the form of persistent capillary bleeding in the same place.

Treatment methods for hemorrhagic diathesis

Treatment of hemorrhagic diathesis is primarily aimed at eliminating bleeding, since they represent an immediate danger to the body. For this purpose, drugs are prescribed that increase blood clotting, and in the case of thrombocytopathy, those that contribute to their better maturation, i.e. aimed at improving metabolism.

Diatheses, which are secondary, are treated along with the disease that caused them. Hemorrhagic diathesis, which are based on hereditary mechanisms, as a rule, cannot be cured, however, constant monitoring and therapy aimed at eliminating symptoms and maintaining health significantly prolongs the life of such patients.

Functional thrombocytopathy in children requires the creation of such conditions for the child that would protect him from serious injury, while at the same time ensuring normal physical activity. You also need good nutrition, prevention of infectious diseases, especially viral etiology.

Hemorrhagic diathesis

Hemorrhagic diathesis - diseases characterized by the presence of a bleeding syndrome (Table 15-1).

Table 15-1.Types of bleeding in some diseases from the group of hemorrhagic diathesis *

THROMBOCYTOPENIC PURPLE

Thrombocytopenic purpura (Werlhof's disease) is a disease characterized by a tendency to bleeding due to thrombocytopenia (a decrease in the platelet count in the blood below 150x10 9 / l) with a normal or increased number of megakaryocytes in the red bone marrow.

Thrombocytopenic purpura is the most common disease from the group of hemorrhagic diathesis. The frequency of detection of new cases of thrombocytopenic purpura is from 10 to 125 per 1 million population per year. The disease usually manifests in childhood. Before the age of 10, the disease occurs

occurs with the same frequency in boys and girls, and after 10 years and in adults - 2-3 times more often in females.

Etiology and pathogenesis

In thrombocytopenic purpura, thrombocytopenia develops due to the destruction of platelets through immune mechanisms. AT to own platelets can appear 1-3 weeks after the transferred viral or bacterial infections; preventive vaccinations; taking medications with their individual intolerance; hypothermia or insolation; after surgery, trauma. In some cases, no specific cause can be identified. Antigens that have entered the body (for example, viruses, drugs, including vaccines) are deposited on the patient's platelets and induce an immune response. Antiplatelet antibodies are predominantly IgG. The reaction "Ag + AT" occurs on the surface of platelets. The life expectancy of platelets loaded with antibodies in thrombocytopenic purpura is reduced to several hours instead of 9-11 days in the norm. Premature death of platelets occurs in the spleen. Bleeding in thrombocytopenic purpura is due to a decrease in the number of platelets, secondary damage to the vascular wall due to the loss of angiotrophic function of platelets, a violation of vascular contractility due to a decrease in the concentration of serotonin in the blood, and the impossibility of retraction of the blood clot.

Clinical picture

The disease begins gradually or acutely with the appearance of hemorrhagic syndrome. The type of bleeding in thrombocytopenic purpura is petechial-spotted (bluish). According to clinical manifestations, two variants of thrombocytopenic purpura are distinguished: “dry” - the patient develops only skin hemorrhagic syndrome; "wet" - hemorrhages in combination with bleeding. Pathognomonic symptoms of thrombocytopenic purpura are hemorrhages in the skin, mucous membranes and bleeding. Cutaneous hemorrhagic syndrome occurs in 100% of patients.

The number of ecchymosis varies from single to multiple.

The main characteristics of cutaneous hemorrhagic syndrome in

thrombocytopenic purpura.

Discrepancy between the severity of hemorrhages and the degree of traumatic impact; their spontaneous appearance is possible (mainly at night).

Polymorphism of hemorrhagic eruptions (from petechiae to large hemorrhages).

Polychromy of skin hemorrhages (color from purple to blue-greenish and yellow, depending on the age of their appearance), which is associated with the gradual conversion of Hb through intermediate stages of decay into bilirubin.

Asymmetry (no favorite localization) of hemorrhagic elements.

Painlessness.

Often there are hemorrhages in the mucous membranes, most often in the tonsils, soft and hard palate. Possible hemorrhages in the eardrum, sclera, vitreous body, fundus.

Hemorrhage in the sclera may indicate the threat of the most severe and dangerous manifestation of thrombocytopenic purpura - hemorrhage in the brain. As a rule, it occurs suddenly and progresses rapidly. Clinically, cerebral hemorrhage is manifested by headache, dizziness, convulsions, vomiting, and focal neurological symptoms. The outcome of cerebral hemorrhage depends on the volume, localization of the pathological process, timeliness of diagnosis and adequate therapy.

Thrombocytopenic purpura is characterized by bleeding from the mucous membranes. Often they are profuse in nature, causing severe post-hemorrhagic anemia that threatens the life of the patient. Children most often experience bleeding from the nasal mucosa. Bleeding from the gums is usually less profuse, but it can also become dangerous during tooth extraction, especially in patients with undiagnosed disease. Bleeding after tooth extraction with thrombocytopenic purpura occurs immediately after the intervention and does not resume after its termination, in contrast to late, delayed bleeding in hemophilia. In girls of puberty, severe meno- and metrorrhagia are possible. Less common are gastrointestinal and renal bleeding.

There are no characteristic changes in the internal organs with thrombocytopenic purpura. Body temperature is usually normal. Sometimes tachycardia is detected, with auscultation of the heart - systolic murmur at the apex and at the Botkin point, weakening of the first tone, due to anemia. An enlarged spleen is uncommon and rather rules out the diagnosis of thrombocytopenic purpura.

Along the course, acute (lasting up to 6 months) and chronic (lasting more than 6 months) forms of the disease are distinguished. At the initial examination, it is not possible to establish the nature of the course of the disease.

Maybe. Depending on the degree of manifestation of the hemorrhagic syndrome, blood parameters during the course of the disease, three periods are distinguished: hemorrhagic crisis, clinical remission and clinical hematological remission.

Hemorrhagic crisis is characterized by a pronounced bleeding syndrome, significant changes in laboratory parameters.

During clinical remission, the hemorrhagic syndrome disappears, bleeding time decreases, secondary changes in the blood coagulation system decrease, but thrombocytopenia persists, although it is less pronounced than during a hemorrhagic crisis.

Clinical and hematological remission implies not only the absence of bleeding, but also the normalization of laboratory parameters.

Laboratory research

A decrease in the content of platelets in the blood up to single ones in the preparation and an increase in bleeding time are characteristic. The duration of bleeding does not always correspond to the degree of thrombocytopenia, since it depends not only on the number of platelets, but also on their qualitative characteristics. The retraction of the blood clot is significantly reduced or does not occur at all. Secondarily (as a result of thrombocytopenia), the plasma-coagulation properties of the blood change, which is manifested by the insufficiency of thromboplastin formation due to a deficiency of the 3rd platelet factor. Violation of the formation of thromboplastin leads to a decrease in the consumption of prothrombin in the process of blood coagulation. In some cases, with thrombocytopenic purpura during the crisis, activation of the fibrinolytic system and an increase in anticoagulant activity (antithrombins, heparin) are noted. In all patients with thrombocytopenia, the concentration of serotonin in the blood is reduced. Endothelial tests (twist, pinch, mallet, prick) during the hematological crisis are positive. In red blood and leukogram (in the absence of blood loss), no changes are found. Examination of the red bone marrow usually reveals a normal or elevated content of megakaryocytes.

Diagnosis of thrombocytopenic purpura is based on a characteristic clinical picture and laboratory data (Table 15-2).

Thrombocytopenic purpura must be differentiated from acute leukemia, hypoor aplasia of the red bone marrow, SLE, and thrombocytopathies.

Table 15-2.Main diagnostic criteria for thrombocytopenic purpura

In hypo- and aplastic conditions, a blood test reveals pancytopenia. The punctate of the red bone marrow is poor in cellular elements.

Blastic metaplasia in the red bone marrow is the main criterion for acute leukemia.

Thrombocytopenic purpura can be a manifestation of diffuse connective tissue diseases, most often SLE. In this case, it is necessary to rely on the results of an immunological study. A high titer of antinuclear factor, the presence of antibodies to DNA indicate SLE.

The main difference between thrombocytopenic purpura and thrombocytopathies is a decrease in platelet count.

Treatment

During the period of a hemorrhagic crisis, the child is shown bed rest with its gradual expansion as the hemorrhagic phenomena fade. Pathogenetic therapy for autoimmune thrombocytopenic purpura includes the appointment of glucocorticoids, immunoglobulin, splenectomy, and the use of immunosuppressants.

Prednisolone is prescribed at a dose of 2 mg / kg / day for 2-3 weeks, followed by a dose reduction and complete withdrawal of the drug. With an increase in the hemorrhagic syndrome during the period of this treatment, the dose of prednisolone can be increased to 3 mg / kg / day for a short time (up to 3 days), followed by a return to the original dose of 2 mg / kg / day. With a pronounced hemorrhagic syndrome, the threat of cerebral hemorrhage, “pulse therapy” with methylprednisolone (20 mg / kg / day intravenously for 3 days) is possible. In most cases, this therapy is quite effective. Initially, the hemorrhagic syndrome disappears, then the platelet content begins to increase. In some patients, after the abolition of glucocorticoids, a relapse occurs.

Currently, in the treatment of thrombocytopenic purpura, intravenous administration of human normal immunoglobulin at a dose of 0.4 or 1 g/kg for 5 or 2 days, respectively (course dose of 2 g/kg) is used with good effect as monotherapy or in combination with glucocorticoids.

Splenectomy or thromboembolization of the vessels of the spleen is carried out in the absence or instability of the effect of conservative treatment, repeated heavy prolonged bleeding, leading to severe post-hemorrhagic anemia, severe bleeding that threatens the life of the patient. The operation is usually performed against the background of glucocorticoid therapy in children older than 5 years, since at an earlier age there is a high risk of post-splenectomy sepsis. In 70-80% of patients, the operation leads to a practical recovery. The rest of the children and after splenectomy need to continue treatment.

Immunosuppressants (cytostatics) for the treatment of thrombocytopenic purpura in children are used only in the absence of the effect of other types of therapy, since the effectiveness of their use is much less than that of splenectomy. Vincristine is used at a dose of 1.5-2 mg / m 2 of the body surface inside, cyclophosphamide at a dose of 10 mg / kg - 5-10 injections, azathioprine at a dose of 2-3 mg / kg / day in 2-3 doses for 1- 2 months

Recently, danazol (a synthetic androgenic drug), interferon preparations (Reaferon, Intron-A, Roferon-A), anti-D-Ig (anti-D) have also been used to treat thrombocytopenic purpura. However, the positive effect of their use is unstable, side effects are possible, which makes it necessary to further study the mechanism of their action and determine their place in the complex therapy of this disease.

To reduce the severity of the hemorrhagic syndrome during the period of increased bleeding, drugs are prescribed that improve the adhesive-aggregation properties of platelets and have an angioprotective effect - aminocaproic acid (contraindicated in hematuria), etamsylate, aminomethylbenzoic acid. To stop nosebleeds, swabs with hydrogen peroxide, epinephrine, aminocaproic acid are used; hemostatic sponge, fibrin, gelatin films.

In the treatment of posthemorrhagic anemia in children with thrombocytopenic purpura, agents that stimulate hematopoiesis are used, since the regenerative abilities of the hematopoietic system are not impaired in this disease. Transfusion of washed erythrocytes, selected individually, is carried out only with severe acute anemia.

Prevention

Primary prevention has not been developed. Secondary prevention is to prevent the recurrence of the disease. Vaccination of children with thrombocytopenic purpura requires an individual approach and special care. Schoolchildren are exempt from physical education; they should avoid sun exposure. In order to prevent hemorrhagic syndrome, patients should not be prescribed drugs that inhibit platelet aggregation (for example, salicylates, indomethacin, barbiturates, caffeine, carbenicillin, nitrofurans, etc.). After discharge from the hospital, children are subject to dispensary observation for 5 years. Shown is a blood test with platelet count 1 time in 7 days, in the future (while maintaining remission) monthly. A blood test is required after each illness.

Forecast

The outcome of thrombocytopenic purpura can be recovery, clinical remission without normalization of laboratory parameters, chronic relapsing course with hemorrhagic crises, and in rare cases, death due to cerebral hemorrhage (1-2%). With modern methods of treatment, the prognosis for life in most cases is favorable.

TROMBASTENIA GLYANTSMANN

Glyantsmann's thrombasthenia is a hereditary disease characterized by a qualitative inferiority of platelets with their normal content and manifested by bruising-type bleeding.

Etiology and pathogenesis. Two types of Glanzmann's disease have been identified: type A (9ΐ or ρ) and type B (9ΐ). The development of the disease is associated with anomalies of glycoprotein IIb / IIIa and a decrease in the activity of glyceraldehyde gophosphate dehydrogenase and pyruvate kinase in platelets, which leads to insufficient thrombus retraction, impaired platelet morphology, decreased ability of platelets to adhere, impaired platelet aggregation; clotting time and platelet count are normal.

Clinical picture Glyantsmann's thrombasthenia is similar to thrombocytopenic purpura. The disease is manifested by bleeding of varying severity.

Diagnosticsbased on clinical and laboratory data. Glyantsmann's thrombasthenia is characterized by:

The presence of increased bleeding in relatives;

Bruising type of bleeding;

Normal content of platelets;

Positive endothelial tests;

Increased duration of bleeding;

Decreased retraction of the blood clot;

Decrease or absence of platelet aggregation with adenosine diphosphate, collagen, adrenaline.

Treatment.In the treatment of Glyantsmann's thrombasthenia, drugs are used that improve the adhesive and aggregation properties of platelets: aminocaproic acid, etamsylate; metabolic agents [eg, trifosadenine (ATP), magnesium preparations]. In addition, calcium chloride, ascorbic acid + rutoside, local hemostatic agents are prescribed.

Prevention.To prevent bleeding, repeated courses of trifosadenine (ATP) and magnesium preparations are recommended after 2-3 months. You should avoid prescribing drugs that worsen the adhesive-aggregating ability of platelets, as well as physiotherapy procedures (UVI, UHF), which can have a damaging effect on them.

Forecast.The disease is incurable, but bleeding may decrease with age.

HEMOPHILIA

Hemophilia is a hereditary disease caused by a deficiency of plasma coagulation factors VIII (hemophilia A) or IX (hemophilia B) and characterized by hematoma-type bleeding. The prevalence of hemophilia is 13-14 cases per 100,000 males.

Etiology and pathogenesis

Depending on the deficiency of factors of the blood coagulation system, two types of hemophilia are distinguished: hemophilia A, characterized by a deficiency of antihemophilic globulin - factor VIII; hemophilia B, accompanied by a violation of blood coagulation due to insufficiency of the plasma thromboplastin component - factor IX. Hemophilia A is 5 times more common than hemophilia B.

Hemophilia A and B (K, recessive) mostly affects men. The pathological X chromosome with the hemophilia gene is transmitted from a sick father to his daughters. They are carriers of the hemophilia gene, passing the disease on to half of the sons who inherit the altered X chromosome, and 50% of their daughters will be carriers (conductors) of the hemophilia gene.

The cause of bleeding in hemophilia is a violation of the first phase of blood coagulation - the formation of thromboplastin due to

with hereditary deficiency of antihemophilic factors (VIII, IX). The time of blood clotting in hemophilia is increased, sometimes the blood of patients does not clot for several hours.

Clinical picture

Hemophilia can appear at any age. The earliest signs of the disease may be bleeding from the bandaged umbilical cord in newborns, cephalohematoma, hemorrhage under the skin. In the first year of life, children with hemophilia may bleed during teething. The disease is more often detected after a year, when the child begins to walk, becomes more active, and therefore the risk of injury increases. Hemophilia is characterized by a hematoma type of bleeding, which is characterized by hemarthroses, hematomas, delayed (late) bleeding.

A typical symptom of hemophilia is bleeding into the joints (hemarthrosis), very painful, often accompanied by high fever. The knee, elbow, ankle joints suffer more often; rarely shoulder, hip and small joints of the hands and feet. After the first hemorrhages, the blood in the synovial cavity gradually resolves, the function of the joint is restored. Repeated hemorrhages in the joints lead to the development of ankylosis. In addition to hemarthrosis, with hemophilia, hemorrhages into the bone tissue are possible with the development of aseptic necrosis, bone decalcification.

Hemophilia is characterized by hematomas - deep intermuscular hemorrhages. Their absorption is slow. The spilled blood remains liquid for a long time, therefore it easily penetrates into the tissues and along the fascia. Hematomas can be so significant that they compress peripheral nerve trunks or large arteries, causing paralysis and gangrene. This causes intense pain.

Hemophilia is characterized by prolonged bleeding from the mucous membranes of the nose, gums, oral cavity, less often the gastrointestinal tract, kidneys. Any medical manipulations, especially intramuscular injections, can lead to heavy bleeding. Tooth extraction and tonsillectomy lead to prolonged bleeding. Hemorrhages in the brain and meninges are possible, leading to death or severe damage to the central nervous system.

A feature of the hemorrhagic syndrome in hemophilia is the delayed, late nature of bleeding. Usually they do not occur immediately after the injury, but after some time, sometimes after 6-12 hours or more, depending on the intensity of the injury and the severity of the disease.

levitation, since the primary stop of bleeding is carried out

platelets, the content of which is not changed.

The degree of insufficiency of antihemophilic factors is subject to fluctuations, which determines the periodicity in the manifestations of bleeding. The severity of hemorrhagic manifestations in hemophilia is associated with the concentration of antihemophilic factors (Table 15-3).

Table 15-3.Forms of hemophilia depending on the concentration of antigens

Diagnosis and differential diagnosis

Diagnosis of hemophilia is based on a family history, clinical picture and laboratory data, among which the following changes are of leading importance.

Increase in the duration of coagulation of capillary and venous blood.

Slow down recalcification time.

Increased activated partial thromboplastin time (APTT).

Violation of the formation of thromboplastin.

Decreased consumption of prothrombin.

Decreased concentration of one of the antihemophilic factors

(VIII, IX).

The duration of bleeding and the content of platelets in hemophilia are normal, tourniquet, pinch and other endothelial tests are negative. The picture of peripheral blood has no characteristic changes, except for more or less pronounced anemia due to bleeding.

Hemophilia is differentiated with von Willebrand's disease, Glanzmann's thrombasthenia, thrombocytopenic purpura (see the relevant sections).

Treatment

The main method of treatment is lifelong replacement therapy. Currently, 3 methods of treating patients with hemophilia can be used: prophylactic, home treatment, treatment after bleeding.

Preventive method - maintaining the activity of the deficient factor at a level of approximately 5%, which makes it possible to prevent hemorrhages in the joints and the patient's disability. High purity antihemophilic globulin concentrates are used. In hemophilia A, drugs are administered 3 times a week; with hemophilia B - 2 times (corresponding to the half-life of antihemophilic globulin). This method allows for complete social adaptation of patients, but is expensive, which limits its use.

Home treatmentinvolves the immediate administration of an antihemophilic drug immediately after an injury or at the slightest sign of an incipient hemorrhage, which allows you to stop bleeding at an early stage, prevent the formation of hematomas, hemarthroses. This method requires training of parents in the use of antihemophilic globulin.

Treatment for bleeding. Antihemophilic drugs are used, the doses of which depend on the nature of the bleeding.

Currently, there is a sufficient number of concentrates of antihemophilic drugs on the Russian market. For hemophilia A, you can use Immunat, Emoklot D.I., Hemophil M, for hemophilia B - Immunin, Aimariks D.I., Octanain.

All antihemophilic drugs are administered intravenously by stream, immediately after they are reopened. Given the half-life of factor VIII (8-12 hours), antihemophilic drugs for hemophilia A are administered 2 times a day, and for hemophilia B (the half-life of factor IX is 18-24 hours) - 1 time per day.

With hemorrhages in the joint in the acute period, complete rest, short-term (3-5 days) immobilization of the limb in a physiological position are necessary. In case of massive hemorrhage, it is recommended to immediately perform a puncture of the joint with aspiration of blood and the introduction of hydrocortisone into the joint cavity. In the future, a light massage of the muscles of the affected limb, the careful use of physiotherapy procedures and therapeutic exercises are shown. In case of development of ankylosis, surgical correction is indicated.

Intensive substitution transfusion therapy in patients with hemophilia can lead to isoimmunization, the development of inhibitory hemophilia. The emergence of inhibitors against coagulation factors VIII and IX complicates treatment, since the inhibitor neutralizes the administered antihemophilic factor, and conventional replacement therapy is ineffective. In these cases, plasmapheresis, immunosuppressants are prescribed. However, a positive effect does not occur in all patients. Complications of hemostatic therapy in

the use of cryoprecipitate and other means also include infection with HIV infection, hepatitis with parenteral transmission, cytomegalovirus and herpes infection.

Prevention

The disease is incurable; primary prevention is not possible. Prevention of bleeding is of great importance. Intramuscular administration of drugs should be avoided due to the risk of hematomas. It is advisable to prescribe drugs orally or intravenously. A child with hemophilia should visit the dentist every 3 months to prevent possible tooth extraction. Parents of a patient with hemophilia should be familiarized with the peculiarities of caring for children with this disease and the principles of providing them with first aid. Since the patient with hemophilia will not be able to do physical work, parents should develop in him a propensity for intellectual work.

Forecast

The prognosis depends on the severity of the disease, the timeliness and adequacy of therapy.

VON WILLEBRAND DISEASE

Von Willebrand's disease (angiohemophilia) is a hereditary disease (type I - 9ΐ, types II and III - ρ), characterized by a bleeding syndrome of a mixed (bruising-hematoma) type.

Etiology and pathogenesis. Von Willebrand disease affects both sexes. The disease is caused by a violation of the synthesis of the plasma-protein complex VIII of the blood coagulation factor (von Willebrand factor), which leads to the pathology of platelet aggregation. There are several types of von Willebrand disease, type III is the most severe. Von Willebrand factor is synthesized in endothelial cells and in megakaryocytes and performs a dual function: it participates in the blood coagulation cascade, determining the stability of factor VIII, and plays an important role in vascular-platelet hemostasis, providing platelet adhesion to the subendothelial structures of the damaged vessel and promoting platelet aggregation.

clinical picture. The first manifestations of the disease in its severe course appear in children in the first year of life. Spontaneous bleeding occurs from the mucous membranes of the nose, mouth, gastrointestinal tract. In puberty, menorrhagia is possible. Often there are subcutaneous hemorrhages in the form of ecchymosis, petechiae, sometimes deep hematomas, hemarthrosis, which makes this disease similar to

hemophilia and distinguishes it from Glanzmann's thrombasthenia and thrombocytopenic purpura. Operations such as tonsillectomy and extraction of teeth are accompanied by heavy bleeding, which can lead to the development of acute post-hemorrhagic anemia. Unlike hemophilia, in von Willebrand's disease, after surgery or injury, bleeding begins immediately, and not after some time, and bleeding does not recur after stopping.

Diagnostics.Diagnostic criteria for von Willebrand disease: family history; mixed type of bleeding; increased bleeding time; a decrease in the concentration of von Willebrand factor and VIII blood coagulation factor; adhesiveness (retention) of platelets and their aggregation under the influence of ristocytin (ristomycin).

Treatment.In the treatment of von Willebrand disease, the same drugs are used as in hemophilia. You can use a synthetic analogue of ADH - desmopressin. In the event of bleeding (except for renal ones!) Aminocaproic acid is used. Mestranol is indicated for menorrhagia.

Lymphogranulomatosis

Lymphogranulomatosis (Hodgkin's disease) is a disease characterized by malignant hyperplasia of lymphoid tissue with the formation of lymphogranulomas in the lymph nodes and internal organs. Lymphogranulomatosis is the most common form of lymphomas. The disease was first described by the English physician Thomas Hodgkin in 1832.

The frequency of lymphogranulomatosis is 1 case per 100,000 children. The peak incidence occurs in preschool age. Boys get sick almost 2 times more often than girls.

Etiology and pathogenesis

The etiology and pathogenesis are not completely clear. A marked increase in the incidence within a certain region and at a certain time indicates that the etiological role may belong to viruses and environmental factors. It is also believed that the Epstein-Barr virus may be the cause of the disease.

The diagnosis of lymphogranulomatosis is established only upon detection of typical ("diagnostic") Berezovsky-Sternberg cells. The nature of the truly malignant cell (Berezovsky-Sternberg binuclear giant cell) remained unknown until the appearance of data on a possible origin from cells of the monocyte-macrophage system, and not from transformed lymphocytes. Lymphogranulomatosis distinguishes the strict spread of the process from one group of lymph nodes to another (unicentric).

Clinical picture

The disease, as a rule, begins with an increase in one or more lymph nodes, often in the cervical group (60-80%). The patient himself or his parents is often the first to notice this. Less often, lymph nodes of other groups are affected (axillary, inguinal, etc.). With the progression of the disease, intrathoracic and intra-abdominal lymph nodes are involved in the process, the liver and spleen increase. Gradually, the size and number of lymph nodes increase, they form a conglomerate of dense, painless, formations that are not soldered to each other and to the surrounding tissues and, according to the figurative expression of A.A. Kissel, reminiscent of "potatoes in a bag." Damage to lung tissue, pleura, gastrointestinal tract, bone marrow, bone skeleton, and nervous system is possible. Common manifestations of lymphogranulomatosis can be fever, night sweats, weight loss, fatigue, drowsiness, loss of appetite, and sometimes skin itching.

Note. Each stage of the disease has two options: A - without intoxication; B - with intoxication.

Laboratory research

In the initial stage of the disease, blood tests reveal moderate neutrophilic leukocytosis, relative lymphopenia, eosinophilia.

liu. ESR is slightly increased. As the disease progresses, leukocytosis is replaced by leukopenia, anemia and thrombocytopenia may occur, which is facilitated by ongoing cytostatic and radiation therapy. ESR reaches high values. Perhaps an increase in the concentration of fibrinogen, a decrease in the content of albumin, an increase a 2 -globulins.

Diagnosis and differential diagnosis

Lymphogranulomatosis should be suspected in the presence of unexplained persistent lymphadenopathy. To clarify the diagnosis and determine the stage of the disease, laboratory, instrumental, x-ray methods of examination are used. However, the decisive factor is the histological examination of the altered lymph node, which reveals specific granulomas, giant multinucleated Berezovsky-Sternberg cells.

Lymphogranulomatosis must be differentiated from banal and tuberculous lymphadenitis, leukemia (see section "Acute leukemia"), malignant tumor metastasis. With the intrathoracic form of lymphogranulomatosis, it is necessary to exclude tuberculous bronchodenitis (see the section "Tuberculosis" in the chapter "Chronic infections"), sarcoidosis, lymphosarcomatosis. With the intra-abdominal variant, tuberculous mesadenitis, a malignant neoplasm of the abdominal cavity, should be excluded.

Treatment

Treatment is carried out in specialized departments. The volume of therapeutic measures depends on the clinical stage of the disease. Currently, preference is given to combination therapy - the simultaneous use of radiation and various schemes of polychemotherapy, which makes it possible to improve long-term results. However, the side effects of therapy must be taken into account. Radiation therapy can lead to damage to the skin, internal organs (radiation pulmonitis, carditis, pneumosclerosis, late hypothyroidism, infertility, etc.). The toxic effect of chemotherapeutic drugs often develops (see the section "Acute leukemia").

Forecast

The prognosis depends on the stage of the disease at the time of initiation of treatment. Modern methods of treatment make it possible to achieve long-term remission, and at stages I and IIA - recovery.

Acute leukemia

Leukemia is a systemic blood disease characterized by the replacement of normal bone marrow hematopoiesis by the proliferation of less differentiated and functionally active cells - early precursors of leukocyte series. The term "leukemia" was proposed in 1921 by Ellerman.

Leukemias are observed with a frequency of 4-5 cases per 100,000 children. In childhood, acute leukemia is diagnosed more often than other cancers. Among all patients with malignant diseases of the hematopoietic and lymphoid tissues, every tenth patient is a child. The peak incidence of leukemia in children occurs at the age of 2-4 years. In connection with the trend towards an increase in the incidence of leukemia observed in recent decades and the continuing high mortality rate, the problem of leukemia in children has become especially relevant for practical healthcare.

Etiology and pathogenesis

The causes of leukemia are not fully understood. At present, the etiological role of ionizing radiation, chemical exogenous factors, oncogenic viruses and the significance of hereditary predisposition to the occurrence of leukemia have been confirmed. The main provisions of the mutation theory and the clone concept are formulated.

The main pathogenetic links are a change in the synthesis of DNA in cells, a violation of their differentiation, and the process getting out of control of regulatory factors. The generally accepted monoclonal theory of the development of hemoblastoses, as well as tumors in general. According to this theory, leukemia cells are a clone - the offspring of a single mutated cell. The clonal theory of the pathogenesis of leukemia considers the disease as a result of the proliferation of an uncontrolled clone of cells that have lost the ability to differentiate and mature, gradually replacing other hematopoietic lineages. Mutations are known to occur almost continuously (on average, one cell mutates every hour). In healthy people, the elimination of altered cells occurs due to the mechanisms of the immune system, which reacts to these cells as if they were foreign. Therefore, the development of leukemia is possible with an unfavorable combination of exposure to mutagenic factors and a weakening of the body's defenses.

Pathomorphology

Leukemia is based on a hyperplastic tumor process in the hematopoietic tissue with foci of leukemic metaplasia in various

ny organs and systems. Most often, pathological hematopoiesis occurs where it existed in the embryonic period: in the spleen, lymph nodes, and liver. The study of blast cells showed that the morphological substrate in acute leukemia in different patients is heterogeneous. According to the morphology of tumor cells, and not according to the duration of the disease, leukemias are divided into acute and chronic.

(Fig. 15-1).

Clinical picture

The disease often begins gradually with the appearance of vague complaints of pain in the bones and joints, fatigue, loss of appetite, sleep disturbance, fever. Seen less often

Rice. 15-1.Forms and variants of leukemia

in children.

sudden onset of the disease with severe intoxication, hemorrhagic syndrome.

The skin and mucous membranes of patients are pale, rarely icteric, sometimes the skin acquires an earthy-greenish hue. Possible gingivitis and stomatitis (from catarrhal to ulcerative necrotic), leukemic infiltration of the skin and mucous membranes. Often note a visible increase in lymph nodes. They have a densely elastic consistency, are painless, and are not soldered to the surrounding tissues. Sometimes Mikulich's symptom complex is determined - a symmetrical increase in the lacrimal and salivary glands due to their leukemic infiltration. In this case, due to the puffiness of the face, the children outwardly resemble patients with mumps.

Hemorrhagic syndrome is one of the most striking and frequent signs of acute leukemia. In patients, hemorrhages in the skin and mucous membranes, bleeding from the nose, gums and gastrointestinal tract, hematuria, hemorrhages in the brain are detected.

Pain in the joints and bones may be due to leukemic infiltration of the synovium, the appearance of periosteal leukemic proliferates, hemorrhages in the joint cavity.

A common symptom of acute leukemia is hepatosplenomegaly. There may be cardiovascular disorders in the form of tachycardia, deafness of heart sounds, functional noise; rarely note the expansion of the boundaries of the heart. It is possible to develop pneumonia.

The defeat of the central nervous system (neuroleukemia) develops as a result of metastasis of blast cells in the nervous system. Most often this occurs in the initial period of the disease due to the fact that the drugs used to treat leukemia do not penetrate the BBB. Most often, neuroleukemia is found in patients against the background of normal hematopoiesis. The clinical picture of neuroleukemia develops gradually. There are dizziness, headache, nausea, diplopia, pain in the spine and intercostal space. Some children have a sharp increase in body weight in a short period of time, bulimia, thirst, which is associated with damage to the diencephalic region. Sometimes neuroleukemia is detected by chance during a clinical examination of patients.

In the course of leukemia, three stages are distinguished (which is necessary to determine the treatment tactics).

Stage I - the debut of the disease, the period from the onset of clinical manifestations to the effect of the therapy.

Stage II - remission. There are complete and incomplete remissions. With complete clinical and hematological remission (duration not

less than 1 month) there are no clinical manifestations, and no more than 5% of blast cells and no more than 30% of lymphocytes are determined in the myelogram. Incomplete clinical and hematological remission is accompanied by normalization of clinical parameters and hemogram, and no more than 20% of blast cells remain in the punctate of the red bone marrow.

Stage III - relapse of the disease. More often, it begins with the appearance of extramedullary foci of leukemic infiltration in the testicles, nervous system, and lungs against the background of normal hematopoiesis. With hematological recurrence, many patients do not present any complaints. In some children, recurrence is diagnosed only on the basis of a study of red bone marrow. Less pronounced symptoms during the period of relapse of acute leukemia are associated with continuous complex treatment that inhibits the development of the disease.

Laboratory research

In acute leukemia in children, blast cells, anemia, thrombocytopenia, high ESR are found in the peripheral blood; the number of leukocytes is variable: leukocytosis of varying degrees is more often detected, less often leukopenia. A characteristic diagnostic criterion is "leukemic dip (gaping)" (Hiatus leucaemicus)- absence of intermediate forms between blast and mature cells. The study of red bone marrow is usually carried out after the study of peripheral blood. A reliable diagnostic criterion for acute leukemia is the detection of blast cells in the sternal punctate in an amount of more than 30% of the cellular composition of the red bone marrow.

Diagnostics

The diagnosis of acute leukemia is made according to clinical data (intoxication, severe pallor, polyadenia and hepatosplenomegaly) and the results of a blood test over time. Identification of blast cells in the hemogram with the presence of "leukemic gaping" makes it possible to suspect acute leukemia. The diagnosis is confirmed by examination of the red bone marrow punctate. In those rare cases when bone marrow examination does not provide clear data for diagnosis, a trephine biopsy is necessary. Detection of diffuse or large-focal blast infiltration in the red bone marrow in violation of the normal ratio of hematopoietic germs helps to make a diagnosis.

To confirm the diagnosis of neuroleukemia, it is necessary to study the cerebrospinal fluid. CSF hypertension, pleocytosis,

persistent increase in protein content. An auxiliary diagnostic method is the study of the fundus. Increasing edema of the optic discs indicates a persistent increase in intracranial pressure associated not only with edema, but also in some cases with leukemoid infiltration of the brain. X-ray examination of the skull reveals compaction of bone tissue along the sutures, increased pattern of finger impressions and vascular pattern, osteoporosis of the Turkish saddle.

Differential Diagnosis

The differential diagnosis of leukemia is primarily carried out with a leukemoid reaction that occurs in response to diseases such as sepsis, severe forms of tuberculosis, whooping cough, tumors, etc. In these cases, hyperleukocytosis is detected in the peripheral blood, but mature cells predominate in the leukogram and only occasionally appear myelocytes, there is no "leukemic gaping". There is no pronounced cell rejuvenation in the punctate of the red bone marrow. Changes disappear as you recover from the underlying disease.

In the differential diagnosis of acute leukemia and agranulocytosis, hypoplastic anemia (see the section "Hypo- and aplastic anemia" in the chapter "Anemia"), thrombocytopenic purpura (see the section "Thrombocytopenic purpura"), collagen diseases, infectious mononucleosis (see the section " Epstein-Barr virus infection" in the chapter "Herpetic infection"), the main criteria should be the results of a cytological examination of the red bone marrow.

Treatment

Therapy of acute leukemia is carried out only in specialized hematological clinics. The task of modern therapy for leukemia is the complete eradication (destruction) of leukemic cells. This is achieved through the use of modern, fundamentally new programs (protocols) for the treatment of acute leukemia in children, including the use of cytostatics, glucocorticoids, radiation therapy, hematopoietic stem cell transplantation, and immunotherapy. There are 4 main areas of therapy:

Specific chemotherapy aimed at achieving and consolidating the remission of the disease; consists of several stages, different for lymphoblastic and myeloid leukemias.

Accompanying maintenance therapy, carried out to reduce intoxication during lysis of the tumor substrate and reduce the side toxic effects of chemotherapy drugs.

Replacement therapy, necessary for threatening thrombocytopenia and severe anemia.

Transplantation of red bone marrow and hematopoietic stem cells.

Stages of treatment: induction (achievement) of remission, consolidation (consolidation) of remission, maintenance therapy. The task of timely therapy is not only to achieve remission, but also to prolong it as much as possible and increase the life expectancy of the patient. Since a large number of leukemia cells remain in the child's body during the period of remission, supportive therapy is necessary. However, neither intensive nor maintenance therapy in most patients is able to restrain the progression of the process, therefore, periodic intensification of treatment is justified - reinduction.

A new method of influencing the leukemic clone is under development - immunotherapy, which consists in the maximum mobilization of the immune system of the patient's body in order to eliminate the immunological inertness of the body in relation to the leukemic agent. They use methods of active immunotherapy based on the introduction of humoral immune factors (plasma, Ig), and methods of adaptive immunotherapy, consisting of the introduction of immunocompetent donor cells into the body (introduction of immune lymphocytes, red bone marrow transplantation). By their nature, immunotherapy methods can also be specific, when other antigens are used to stimulate the immune response, for example, the BCG vaccine and the smallpox vaccine. Rational schemes of immunotherapy are still being worked out.

The success of the treatment of acute leukemia depends both on the appointment of specific therapy and measures for the prevention and treatment of complications caused by various (mainly infectious) diseases and side effects of cytostatics. During treatment, patients may experience nausea, vomiting, anorexia. The most formidable complication of cytostatic therapy is the suppression of bone marrow hematopoiesis, as a result of which infectious complications such as pneumonia, enteropathy, ulcerative necrotic stomatitis, purulent otitis media, etc. develop in patients. During this period, it is necessary to carry out intensive replacement therapy, use broad-spectrum antibiotics. With deep myelosuppression, in order to prevent infectious complications, the patient is placed in a separate ward or box, where conditions are created that are as close to sterile as possible. The staff follows the same aseptic and antiseptic rules as in operating units.

The treatment of patients with acute leukemia presents significant difficulties: it is always lengthy and often fraught with severe complications. In this regard, treatment must be carried out in specialized departments with full understanding between the doctor and the parents of the sick child. Achievements of recent years in the treatment of acute leukemia can significantly prolong the life of the child, and in some patients - to achieve complete recovery.

Pathogenesis

Epidemiology

Clinic

Hemorrhagic diathesis caused by a defect in the vascular wall - congenital - acquired 4. Hemorrhagic diathesis caused by excessive fibrinolysis - endogenous (primary and secondary) - exogenous 5.

Hemorrhagic diathesis caused by a combination of disorders of various components of the hemostasis system (Willebrand's disease, DIC, etc.) This classification does not include all known hemorrhagic diathesis.

There are more than 300 of them. It is a scheme of principles for classifying hemorrhagic conditions, following which it is possible to classify not only any of the known hemorrhagic conditions, but also each newly discovered one.

Thrombocytopathy is the second group of hemorrhagic conditions caused by the inferiority of the platelet component of hemostasis. It combines diseases that are manifested by a qualitative inferiority of platelets with the preservation of their number.

She received the name thrombocytopathy. In recent years, there have been major changes in the classification of thrombocytopathies.

Their essence lies in the fact that many nosological forms, a characteristic feature of which was bleeding, turned out to be heterogeneous. Attempts to link one or another feature of functional disorders of platelets with damage or features of the development of other organs or systems (Hermansky-Prudlak syndrome, Chediak-Higashi, etc.)

) in this regard also demonstrate a certain polymorphism. All this forced doctors to focus on the specific pathology of platelet function, which formed the basis.

The following types of thrombocytopathies are distinguished: 1) thrombocytopathy with impaired platelet adhesion; 2) thrombocytopathy with impaired platelet aggregation: a) to ADP, b) to collagen, c) to ristomycin, d) thrombin, e) adrenaline; 3) thrombocytopathy with impaired release reaction; 4) thrombocytopathy with a defect in the "accumulation pool" of released factors; 5) thrombocytopathy with retraction defect; 6) thrombocytopathy with a combination of the above defects. In addition to stating platelet defects, it is necessary to supplement the diagnosis of the disease with the obligatory indication of the quantitative side of the platelet link (hypothrombocytosis, hyperthrombocytosis, normal platelet count), as well as the statement of concomitant pathology.

The classification of vascular diseases that occur with hemorrhagic manifestations suggests their division depending on the localization of the lesion of the morphological structures of the vessel. Distinguish diseases with damage to the endothelium itself and diseases with damage to the subendothelium.

Endothelial lesions are divided into congenital and acquired. A representative of congenital damage to the endothelium is hereditary hemorrhagic telangiectasia (Rendu-Osler disease).

Among the acquired lesions of the endothelium, diseases of an inflammatory and immune nature, damage caused by mechanical factors are distinguished. Inflammatory and immune acquired hemorrhagic conditions are Henoch-Schonlein disease, nodular arteritis, allergic granulomatosis, vasculitis in infectious diseases and drug exposure.

The same subgroup includes chronic inflammatory infiltrates, such as Wegener's granulomatosis, temporal arteritis, Takayasu's arteritis. Among the mechanical damage to the endothelium, orthostatic purpura and Kaposi's sarcoma are distinguished.

Hemorrhagic diseases caused by disorders of subendothelial structures are also divided into congenital and acquired. Among the congenital, there are Euler-Danlos syndrome, elastic pseudoxanthoma, Marfan syndrome, as well as osteogenesis imperfecta disease.

Hemorrhagic conditions in amyloidosis, senile purpura, corticosteroid purpura, simple purpura, and hemorrhagic conditions in diabetes mellitus are combined into acquired defects of the subendothelium. Approximate wording of the diagnosis: 1.

Immune thrombocytopenic purpura, occurring with hemorrhages on the skin and on visible mucous membranes, gingival, nasal, intestinal bleeding. 2.

Hemophilia A (classic hemophilia) caused by a deficiency of factor VIII with hemorrhages in the muscles and joints, nasal, gingival, intestinal, uterine bleeding. 3.

Disseminated intravascular coagulation syndrome with skin petechiae, bleeding of mucous membranes, hematuria, hemoptysis. Bleeding caused by a quantitative or qualitative defect in platelets has certain characteristics.

Most often, it is characterized by skin lesions - the appearance of small, from a point to a pinhead, skin hemorrhages that occur with minimal bruises, or, as it were, spontaneously, called petechiae. But along with them, bruises and bruises of large sizes may appear - ecchymosis, resulting from soaking the skin and mucous membranes with blood.

They easily occur in places of pressure on the skin of elastic bands, belts, etc.

Arising at different times, petechiae and ecchymosis, undergoing stages of natural development and changing their color from purple-blue to blue, blue-green, green-yellow, etc.

They lead to the formation of the so-called "leopard skin" in the patient. Multiple superficial petechiae and ecchymosis do not disappear with pressure and often form in places with increased capillary pressure.

Superficial cuts and scratches are accompanied by prolonged bleeding. Along with skin manifestations, hemorrhages on the mucous membranes are characteristic of a platelet defect.

Very frequent nosebleeds from the Kisselbach area, gingival bleeding, provoked by the use of a toothbrush. Petechiae and hemorrhagic blisters often appear on the mucous membranes of the cheeks, reaching large sizes when the mucous membrane of the cheeks is injured during chewing.

Surgical interventions on the organs of the oral cavity and in the nasopharynx are very dangerous. Extractions of teeth and removal of the tonsils can lead to massive bleeding that threatens the life of the patient.

It is curious that these patients tolerate abdominal operations much easier. In the pathology of the platelet link of hemostasis, hemorrhages in the abdominal cavity, pleura, eye cavity, retina, and brain hemorrhages are described.

Pulmonary, intestinal and renal bleeding is also not uncommon. In women, the main manifestations are often menorrhagia and metrorrhagia - prolonged heavy menstruation and extracyclic uterine bleeding.

Cases of hemorrhages in the ovaries, simulating the development of an ectopic pregnancy, are described. For defects in the platelet link of hemostasis, hemorrhages in the joints and muscles are not characteristic.

Most often, such hemorrhagic diathesis develops due to a decrease in the number of platelets. It is generally accepted that the condition is considered critical when the platelet level is below 30 * 109 / l, although some authors believe that bleeding is only necessary to reduce platelets to even lower numbers - 7 * 109 / l.

When explaining documented thrombocytopenia, it must always be remembered that in every healthy person up to 1/3 of all platelets are deposited in the spleen. With an increase in the size of the spleen, the number of deposited platelets can increase significantly and lead to a decrease in their number in the peripheral blood.

In such cases, it is often necessary to decide whether a splenectomy is necessary, in most cases returning the platelet count to normal. With the use of large doses of alcohol, thrombocytopenia occurs both due to the destruction of platelets, and by reducing their formation.

Like anemia, thrombocytopenia can be dilutional in nature, i.e.

Appear due to dilution of blood. It must be admitted that such situations are not rare, but they are of no practical importance, since they do not cause hemorrhagic manifestations and are of a transient nature.

Of practical importance are thrombocytopenia that occurs when a large blood loss is replenished with "old" blood. This should be taken into account and the transfusion of "old" blood should be alternated with fresh.

In addition to thrombocytopenia, caused by insufficient reproduction, shortening of life, deposition and dilution of platelets, it is necessary to keep in mind the possibility of their development due to the elimination of platelets from the bloodstream when settling on the surfaces of heart-lung machines and settling into the resulting blood clots, especially with DIC. In addition to the quantitative defect of platelets, their qualitative disorders are also known - thrombocytopathies.

The existence of thrombocytopathy became known after the publication of the observations of the Swiss doctor E. Glanzman, in 1918.

Described the patient, whose hemorrhagic manifestations corresponded to thrombocytopenic purpura, although the number of platelets was within the normal range. The clinical picture of hemophilia is characterized by bleeding, which is usually associated with some kind of trauma, both domestic and surgical.

More often, bleeding develops some time after tissue damage and is characterized by difficulty in stopping it. Bleeding can be external, subcutaneous, intramuscular, intraarticular and parenchymal.

The most traumatic are hemorrhages in the muscles and joints. Hemorrhage into the cranial cavity often ends in death.

Nose and gingival bleeding, mucosal hemorrhage, umbilical bleeding of newborns, metrorrhagia, bleeding from the gastrointestinal tract and urinary tract can occur with any variant of hemophilia. In some patients, bleeding can be provoked by infections that cause local inflammation (tonsillitis, cystitis, acute respiratory infections, etc.).

It is important to keep in mind that the severity of hemorrhagic manifestations only in hemophilia A and B has a certain correlation with the level of procoagulant defect. With other hemophilias, this cannot be clearly traced.

Differences in the clinic have only two diseases caused by a defect in fibrin formation. They are a defect in factor XIII, which is characterized by the formation of rough keloid scars at the sites of tissue damage, as well as a defect in the factor VIII antigen (von Willebrand disease).

This disease was described by the Finnish physician Erich von Willebrand among the inhabitants of the Åland Islands in 1926. In the study of these patients, it was shown that normal platelets are not able to perform their hemostatic function without the factor VIII antigen that attaches them to the damaged area of ​​the vascular wall.

This was the reason for the "thrombocytopenic" clinical picture - petechial bleeding into the skin and mucous membranes - nose and mouth bleeding, ecchymosis, menorrhagia, prolonged bleeding from cuts, with extreme rarity of deep hematomas and hemarthrosis. von Willebrand's disease, inherited in an autosomal dominant manner, which affects both men and women, is the third most common hemorrhagic condition caused by a defect in procoagulants, accounting for about 10% of their number.

At the same time, the peculiarity of the clinical picture often raises objections to the unconditional attribution of von Willebrand's disease to the category of hemophilia. The works of recent years give grounds to talk about the heterogeneity of this disease and the possibility of distinguishing six of its subtypes.

Deciphering the pathogenesis of von Willebrand's disease showed the possibility of developing the disease, both with an absolute decrease in the factor, and in the presence of its functional inferiority. Hemorrhagic conditions caused by a combination of violations of various components of the hemostasis system are very common.

The most typical representative of this group is the acute and subacute variants of disseminated intravascular coagulation syndrome (DIC). DIC, also called thrombo-hemorrhagic, or consumption coagulopathy, has its own developmental features.

Hemorrhages are secondary. They are caused by multiple microthrombi that consume fibrin and platelets and thereby cause hypocoagulation.

Hypocoagulation is usually enhanced by hyperfibrinolysis, which is reactive in nature. Chronic types of DIC are practically not manifested by bleeding and will not be considered in this section.

Clinically, disseminated intravascular microcoagulation manifests itself in a variety of ways. This diversity is determined by an unpredictable and most bizarre combination of the prevalence and severity of thrombosis of the microcirculatory pathways of various internal organs with the activation of the kinin system, complement, and secondary fibrinolysis.

Of significant importance is not only the intensity of thrombosis in its quantitative terms, but also the rate of thrombus formation, as well as the localization of the predominant obstruction of the microcirculatory bed, which determines the dysfunction of certain organs. Hemorrhages, a decrease in blood pressure and a decrease in the functionality of internal organs are determined by the severity of intravascular microcoagulation.

Bleeding and bleeding into the skin are often the most noticeable symptoms of DIC, especially in young people. They are determined by such changes in blood properties as a decrease in the level of procoagulants, a decrease in the number of platelets, changes in their functional properties caused by the action of thrombin, as well as activation of reactive fibrinolysis and the action of some fibrin degradation products - fibrinogen.

Bleeding is most often manifested by skin petechiae, ecchymosis, bleeding of mucous membranes, bleeding from injection sites, hematuria, hemoptysis. Sometimes bleeding and microthrombosis can be limited to one or more organs, such as the brain, kidneys, and lungs.

At the same time, the phenomena of functional insufficiency of one or another organ, which determine the clinical symptoms, come to the fore. The course of DIC may also have a reverse development, however, this depends both on the treatment and on the ability of the body itself to neutralize active blood coagulation factors, fibrin.

The clinical picture of intravascular microcoagulation may be complicated by the phenomena of acute organ ischemia. These cases cannot be considered rare.

Quite often, this phenomenon is observed in patients with neoplastic diseases, in whom signs of DIC can be combined with symptoms of superficial phlebitis, deep vein thrombosis, lower leg, arterial thrombosis and abacterial thrombotic endocarditis. Although the clinical manifestations of disseminated intravascular microcoagulation are very diverse, it must be borne in mind that all of them are primarily caused by impaired blood flow in the microcirculatory bed of certain organs due to microthrombosis, causing one or another violation of their functions, and secondly, by the severity and prevalence hemorrhagic diathesis, which in turn can affect the functions of certain organs and systems, modifying the clinical picture of the disease in its own way.

The possibility of developing DIC should be remembered when the patient has circulatory disorders, infections, malignant neoplasms, hemolytic anemia. The clinical picture, quite clearly, can help diagnose intravascular microcoagulation in an acute form.

To identify the chronic type of DIC, clinical indicators provide much less information. Hemorrhagic conditions caused by the pathology of the vascular wall are a very heterogeneous group, which is characterized by a very wide range of clinical manifestations.

The most common manifestations of these diseases are skin petechiae and hemorrhagic rashes, easily induced or spontaneous bleeding from mucous membranes of various localization. About vascular lesions as a cause of hemorrhagic syndrome, one can speak only in the absence of pathology from platelets and the process of fibrin formation.

Let's take a closer look at the most common of the above. The most common congenital vascular pathology inherited in an autosomal dominant manner is hemorrhagic telangiectasia (Randu-Osler).

The diagnosis becomes in the presence of the classic triad - skin telangiectasias, the hereditary nature of the disease and frequent bleeding. Capillaries and postcapillary venules are affected.

Telangiectases can range in size from pinpricks to large angiomas 3–4 mm in diameter. They are located on the mucous membranes, skin of the face, trunk and upper limbs.

Their recognition is not difficult. Up to 20% of patients with this pathology have arteriovenous shunts in the lungs.

Usually the disease proceeds benignly with timely measures to stop bleeding and treat anemia. The presence of telangiectases is determined by the absence of an elastic membrane and muscle fibers in certain parts of the vessels.

The wall consists only of endothelium. In other areas, the formation of arteriovenous aneurysms is noted.

The disturbed structure does not allow the vessels to contract when damaged, which determines bleeding.

Differential Diagnosis

The prolongation of bleeding time with normal values ​​of prothrombin and partially activated thromboplastin time makes one think about the pathology of the platelet link. Platelet counting allows to separate thrombocytopathies from thrombocytopenias. Prolongation of partially activated thromboplastin time with normal prothrombin and bleeding time suggests the most common hemophilia. Prolongation of prothrombin time with normal bleeding time and partially activated thromboplastin time makes it possible to diagnose a factor VII defect (hypoproconvertinemia).

The addition of an easy-to-perform study using barium plasma to the listed tests makes it possible to differentiate hemophilia A from hemophilia B, which is important for the selection of therapeutic measures. Naturally, this diagnosis is only qualitative. Determination of the quantitative severity of the procoagulant defect requires tests using plasmas, which is carried out in specialized laboratories. In the same place, tests are performed that decipher defects in the functions of platelets - adhesion, aggregation, release reactions, retractions.

For practitioners, high-quality diagnostics is quite enough, in cases where the clinical situation requires urgent therapeutic measures.

Treatment

AGG concentrates, fresh frozen plasma, concentrates containing factor IX are used. In recent years, the effectiveness of the use of desmopressin in these patients has been shown, which is able to rapidly increase plasma levels of factor VIII complex due to its release from vascular endothelial cells.

The drug (0.3 mg/kg) is injected intravenously over 15-30 minutes. Treatment of von Willebrand's disease The use of cryoprecipitate is recommended.

Desmopressin may be effective in type I von Willebrand disease. Treatment of immune thrombocytopenic purpura.

Splenectomy is recommended for patients with a disease duration of more than 1 year in the presence of 2-3 exacerbations after corticosteroid therapy. This method is also indicated for all patients with severe purpura in the absence of the effect of the use of corticosteroids.

With the ineffectiveness of corticosteroid therapy and splenectomy, it is possible to use cytotoxic drugs (vincristine 1.4 mg/m2 or vinblastine 7.5 mg/m2) intravenously once a week for 4-6 weeks. Treatment of telangiectasia (Rendu-Osler disease).

There is no specific therapy. With a daily blood loss of 50-100 ml, the use of iron preparations, blood transfusions are recommended.

In case of bleeding due to defects in prothrombin complex factors, the use of vitamin K (a synthetic water-soluble drug, 5 mg daily) is recommended. Treatment of DIC.

Due to the pronounced heterogeneity of this group, as well as the lack of a generally accepted classification of such conditions, it is not possible to claim the possibility of giving exhaustive recommendations for each specific case. Nevertheless, we believe that the principles of treatment of such patients should be known to every physician.

1. Removal or active therapy of the main cause of DIC - antibiotics, cytostatics, active treatment of shock, normalization of circulating plasma volume, delivery, hysterectomy, etc.

Stopping intravascular coagulation - the introduction of heparin subcutaneously or intravenously, antiplatelet drugs (curantil, ticlopedin, acetylsalicylic acid, etc.); administration of vitithrombin III concentrate; the introduction of protein concentrate "C".

3. The introduction of blood components according to indications - platelet mass, washed erythrocytes, cryoprecipitate, prothrombin complex; fresh frozen plasma.

4. Suppression of excessive fibrinolysis - g-aminocaproic acid, para-aminobenzoic acid.

Naturally, this treatment should be carried out with constant monitoring of the level of fibrinogen, platelet count, and fibrinolysis. Extracorporeal gravisurgical correction of the aggregate state of blood in hematological and non-hematological diseases.

Gravitational blood surgery (GCC) is a complex of extracorporeal methods for correcting the composition of peripheral blood using special devices that use gravitational forces to separate fluid into fractions. In these devices, called hemoprocessors-fractionators, under the influence of centrifugal forces, the blood is separated into components of various molecular weights.

As a result, it is possible to remove cells, plasma, toxic substances, immune and other blood components, as well as to replace them with drugs that change its morphological composition, state of aggregation and rheological properties. Depending on the composition of the morphological substrate to be removed (apheresis), HCC methods are divided into several varieties: 1) plasmapheresis - removal of plasma from peripheral blood; 2) granulocytapheresis - removal of granulocytes; 3) lymphocytapheresis - removal of lymphocytes; 4) plateletpheresis - removal of platelets; 5) blastocytapheresis - removal of blast cells; 6) lymphapheresis - removal of lymph from the thoracic lymphatic duct; 7) myelokaryocytepheresis - separation of bone marrow suspension into cellular elements and extraction of its elements from the suspension.

The HCC methods restore the normal composition of the blood not only through a combined supplement (transfusion), but also by removing (apheresis) individual components. various pathological conditions. Special devices have been designed for the MCC operation.

In devices with a continuous blood flow, the fractionation process is carried out continuously, during the operation the blood is divided into components, the necessary fraction is withdrawn, and the rest of the blood is continuously returned to the patient. The devices have a centrifuge rotor, a system of highways, roller pumps, an engine, a control system.

Under the action of peristaltic pumps, blood from the patient through the catheter is supplied to the system of highways, where it is mixed with an anticoagulant and enters the rotor of the device. In the rotor, blood is fractionated into components under the influence of centrifugal forces (gravitational forces), layers of various fractions are brought to special holes, and the necessary blood component is removed using peristaltic pumps.

The blood is continuously reinfused. In devices with intermittent blood flow in the rotor, its fractionation occurs.

As blood accumulates in the rotor, plasma is sequentially displaced from it, then platelets and leukocyte suspension. After that, the flow of blood from the patient is stopped and the erythrocyte suspension is fed into the reservoir for reinfusion by the reverse motion of the rotor.

Following the reinfusion of red blood cells, the cycle repeats. The equipment provides a series of methodological techniques: 1) deglycerinization of thawed erythrocytes; 2) washing of preserved erythrocytes; 3) erythrocytepheresis; 4) plasmapheresis; 5) lymphocytapheresis; 6) granulocytapheresis; 7) plateletpheresis; 8) plasma sorption on biological sorbents (hepatocytes, spleen cells, pancreatic beta cells); 9) plasma sorption on chemical sorbents; 10) oxygenation of erythrocytes against the background of erythrocytepheresis.

Gravisurgical operations are used in the following cases associated with violations of the morphological and biochemical composition of the blood: 1) correction of the protein composition of the plasma - removal of exogenous and endogenous pathological proteins; 2) correction of immune disorders - removal from the blood of circulating immunoglobulins, antigen-antibody complexes, complement components, T-lymphocytes, lymph from the thoracic lymphatic duct; 3) correction of the cellular composition of the blood - removal of platelets, erythrocytes, blast cells, etc. In most cases, gravisurgical operations are performed when patients are resistant to conventional therapy and need measures to reduce this resistance and increase the effectiveness of treatment with conventional methods of conservative therapy.

Gravisurgical correction of the protein composition of the plasma is used for pathological conditions and diseases indicated in the table. Gravity plasmapheresis can be combined with other methods to remove abnormal proteins from plasma.

Most often, immunosorption, plasma sorption are connected to plasmapheresis. The most successful operation of gravisurgical correction of the plasma composition is when it is necessary to stop the syndrome of prolonged compression, increased viscosity, disseminated intravascular coagulation.

Methods of gravisurgical correction are promising and effective also, if necessary, to actively regulate the aggregate state of the blood, disturbed as a result of various diseases or as a result of surgical intervention using a heart-lung machine. Controlling the rate of supply of infusion solutions in the line, the return of the erythrocyte mass to the patient on the devices PF-0.5 or RK-0.5 allows you to regulate the filling of the vascular bed, maintain blood pressure at the required level, simultaneously remove cholesterol, fibrinogen and other substances, significantly improve rheological blood properties and microcirculatory blood flow, including thromboembolism of small branches of the pulmonary artery.

With the help of gravisurgical correction of the aggregate state of blood, it is possible to form controlled hemodilution, hypo- and normovolemia, to control the concentration of any biochemical factors in the blood and the number of its cellular elements. A new approach to antithrombotic therapy consists in the use of gravisurgical fractionation of blood in a continuous flow using devices of the PF-0.5 type to reduce coagulation processes and reduce blood viscosity with the removal of an excess amount of cellular and plasma hemostasis factors from the circulating blood.

At the same time, rheologically active solutions (rheopolyglucin, albumin) and antithrombin III as part of fresh frozen donor plasma are introduced into the vascular bed. The use of this method does not exclude traditional antithrombotic agents, but, on the contrary, increases their therapeutic effect, expands the possibilities of modern cardiology, resuscitation, and surgery.

With the syndrome of prolonged compression, plasmapheresis is indicated for all patients who have signs of intoxication, the duration of compression is more than 4 hours, and pronounced local changes in the damaged limb. Effective correction of the human immunological status through plasmapheresis, removal of antibodies and immune complexes from the bloodstream, immunogenic plasma proteins and inflammatory mediators - kinins, complement factors that determine the inflammatory response and tissue damage in autoimmune processes.

The traditional method of reducing immunological reactivity with immunosuppressive pharmacological agents (cyclophosphamide, azathioprine, corticosteroids) in diseases called "immune complex diseases" is not always effective, and the drugs themselves are quite toxic. Clinical observations indicate a significant therapeutic efficacy of gravitational plasmapheresis in this kind of diseases.

Pathological conditions Diseases Alloimmunization Hemolytic disease of the newborn Kidney transplant rejection Autoimmunization Autoimmune hemolytic anemia Autoimmune thrombocytopenic purpura Myasthenia gravis Goodpasture's syndrome Pemphigus Aggression of immune complexes Systemic lupus erythematosus Tolerability of cytapheresis in the vast majority of cases is good, which allows this operation to be performed on an outpatient basis, detaining patients after the session doctor for 2-4 hours. In chronic lymphocytic leukemia, during one session of leukocytapheresis, up to 4 * 1012 leukocytes are removed from the patient's body.

The number of removed leukocytes is directly dependent on the initial peripheral blood leukocytosis. The intervals between sessions and courses of cytopheresis, their number are determined by the characteristics of the patient's response to this operation and the clinical and hematological picture of the disease.

The clinical effect of gravitational cytapheresis makes it possible to carry out subsequent cytostatic treatment and improve the response to it, which is important in cases of development of resistance to chemotherapy in tumor diseases of the blood system. Methods for correcting the cellular composition of blood based on the transfusion of missing cellular elements are also used in clinical practice.

Transfusions of individual cell concentrates obtained from donors by cytapheresis sometimes occupy a leading place Pathological conditions Diseases of hemoblastosis Acute leukemia Chronic lymphocytic leukemia Chronic myelogenous leukemia Erythremia Thrombocytosis Hemorrhagic thrombocythemia Thrombocytosis Hemoglobinopathies Sickle cell anemia beta-thalassemia Chronic non-allergic pathology of the lungs Chronic pneumonia Bronchial asthma (infectious form) Other conditions Rheumatoid arthritis Glomerulonephritis Vasculitis Malignant neoplasms Inflammatory bowel disease (Crohn's disease) To obtain a stable positive effect, repeated use of intensive plasmapheresis in combination with immunosuppression and cytostatic therapy is necessary. The result in each case will depend on the timing of the start of treatment, the frequency and volume of plasmapheresis and plasma exchange, a clear establishment of patient selection criteria.

Methods for correcting the cellular composition of blood based on cytapheresis are used in the treatment of hemoblastoses, thrombocytosis, hemoglobinopathies and other diseases in the complex therapy of a number of diseases. Transfusion of granulocytes is used in the treatment of patients with burns, sepsis, neutropenia.

Platelet transfusions are used for hemorrhages of thrombocytopenic origin. The erythrocyte mass, free from impurities of leukocytes and platelets, is transfused to patients with antileukocyte antibodies during kidney transplantation, prolonged compression syndrome and surgical interventions.

When establishing contraindications for HCC surgery, it is important to determine the degree of somatic compensation of patients, the state of the cardiovascular system, the system of regulation of the aggregate state of blood, hemograms, liver and kidney functions. In case of peptic ulcer of the stomach and duodenum in the acute stage, mental illness, HCC operations are not recommended.

It is mandatory to determine the possible presence of surface antigen and antibodies to the human immunodeficiency virus (HIV). In case of detection of HAsAg or antibodies to HIV, it is necessary to use specially dedicated blood fractionation devices for carrying out a gravisurgical operation.

Attention! The described treatment does not guarantee a positive result. For more reliable information, ALWAYS consult a specialist.

Hemorrhagic diathesis

The group of hemorrhagic diathesis includes diseases that are characterized by an increased tendency to hemorrhage and bleeding.

Considering which of the links of hemostasis is impaired, vasopathy, thrombocytopathy, and coagulopathy are distinguished.

Vasopathy. At the heart of vasopathies is the defeat of the vascular wall of capillaries and small blood vessels. They unite a large group of congenital and acquired disorders of the vascular wall (multiple Randu-Osler angiomatosis, isolated angiomatosis, hemorrhagic vasculitis, scurbut, Mayocchi purpura, etc.). Currently, of these diseases, hemorrhagic vasculitis is the most common.

Thrombocytopathy- this is a disease of bleeding, caused either by an insufficient number of platelets in the blood (thrombocytopenic purpura), or a violation of their functions (Glantzmann's thrombosthenia, von Willebrand-Jurgens angiohemophilia). Bleeding is due to a lack of platelet factor of the hemostasis system. The most common disease in this group is thrombocytopenic purpura.

Coagulopathy. This group includes bleeding diseases associated with a deficiency of plasma coagulation factors. The most pronounced changes in blood coagulation occur with a deficiency of factors involved in the 1st phase of the formation of active thrombokinase. Pathological processes caused by a deficiency of these factors are called hemophilia (A, B, C, D). A coagulation disorder resulting from a deficiency of factors involved in subsequent phases is called pseudohemophilia.

Hemorrhagic vasculitis (Schonlein-Genoch disease, capillary toxicosis)

Etiology. This is an infectious-allergic disease, which is characterized by damage to the vascular wall of small blood vessels with the formation of microthrombi.

Allergens that cause sensitization of the body can be: bacterial infection (scarlet fever, tonsillitis, SARS); medicines (vaccines, antibiotics, B vitamins, etc.); food products (obligate allergens: eggs, fish, strawberries, citrus fruits, food products containing preservatives). In many patients it is possible to detect foci of chronic infection.

In all cases, there is a latent asymptomatic period of 1-3 weeks, which corresponds to the time required for the formation of antibodies.

Clinic. The disease often begins acutely. In most children, body temperature rises to 38–39 ° C, symptoms of intoxication appear. In the clinic of the disease, several leading syndromes are distinguished.

Skin hemorrhagic syndrome- first on the lower extremities and buttocks, then on the upper extremities, lower back, less often on the chest, neck, face, small erythematous spots appear, which quickly become hemorrhagic. Hemorrhagic rash symmetrical, grouped around the joints, on the buttocks, extensor surfaces of the limbs. Rashes are polymorphic due to further “spills”. Sometimes there may be phenomena of angioedema, as well as swelling of the hands, feet, legs, eyelids, face.

Articular syndrome accompanied by damage to large joints, reversibility of the process and complete restoration of joint function. Flying changes.

Abdominal syndrome characterized by sudden cramping, very sharp pains, which are often localized around the navel. The clinical picture in such cases resembles an "acute abdomen". In severe cases, there may be vomiting with an admixture of blood, bloody stools.

renal syndrome observed less frequently than others. It joins after 1-3 weeks of illness. Moderate hematuria and proteinuria are noted, which disappear during treatment.

Diagnosis. In the analysis of peripheral blood, leukocytosis, neutrophilia, increased ESR, and eosinophilia are detected with varying degrees of severity. It is necessary to count the number of platelets (possible DIC). The duration of bleeding and the time of blood clotting are not changed. Endothelial tests are negative. Given the possible damage to the kidneys in all patients, it is necessary to systematically examine the urine.

Treatment carried out in a hospital. If a connection with the infection is established, a course of antibiotics is indicated. Assign desensitizing agents, drugs that strengthen the vascular wall, antiplatelet agents. If necessary, carry out symptomatic therapy.

Diet in the acute period with the restriction of animal proteins, salt, extractives. Bed rest for 2-3 weeks, then it is gradually expanded, since relapses of purpura are possible. It is expedient for everyone to prescribe activated charcoal, enterosorbent, polypephane inside.

The children have been in the dispensary for 5 years. They are exempted from vaccinations for 2 years.

Thrombocytopenic purpura (Werlhof's disease)

Etiology. A disease caused by quantitative and qualitative insufficiency of the platelet link of hemostasis. There are congenital forms of thrombocytopenia and acquired. The genesis of the primary (idiopathic) acquired form of Werlhof's disease is not well understood. In a special group, thrombocytopenic purpura of newborns born to mothers with Werlhof's disease, or mothers sensitized with fetal platelets, is distinguished.

Past infections (SARS, measles, rubella, etc.), preventive vaccinations, physical and mental traumas, and other external factors can lead to platelet deficiency. The vascular endothelium, deprived of the angiotrophic function of platelets, undergoes dystrophy, which leads to an increase in vascular permeability and spontaneous hemorrhages.

Clinic. The main symptoms of the disease are bleeding into the skin and bleeding from the mucous membranes, which occur either spontaneously or due to minor injuries. Hemorrhages are located on the trunk, limbs, oral mucosa, less often on the face and scalp. A feature of the hemorrhagic rash is their disorder, asymmetry, polymorphism and polychromy (of different colors - from reddish-bluish to green and yellow). Along with the petechial rash, there are usually bruises of various sizes.

Typical symptoms are bleeding from the nose (profuse), from the oral cavity (gums, tongue, tonsillectomy, during tooth extraction), from internal organs (gastrointestinal, renal, uterine).

Diagnosis. The most typical laboratory abnormalities are thrombocytopenia, anemia, increased bleeding time, and positive endothelial tests. Blood clotting is normal.

Treatment hemorrhagic syndrome includes the appointment of local (fibrin sponge, fresh plasma, hemostatic sponge, thrombin) and general (epsilon-aminocaproic acid, dicynone, adroxan, platelet mass) hemostatic agents. Assign desensitizing agents, drugs that strengthen the vascular wall. Plasmapheresis has a good effect. Drugs are administered orally or intravenously whenever possible. It is necessary to avoid manipulations associated with damage to the mucous membranes and skin. In severe cases, resort to hormonal therapy. Treatment is carried out in a hospital.

Dispensary observation in the acute form of the disease is carried out for 5 years, in the chronic form - until the transition of the child to an adult clinic.

Hemophilia BUT

Etiology. A hereditary disease characterized by a sharply slow blood clotting and increased bleeding due to insufficient coagulation activity of VIII (hemophilia A) plasma coagulation factor.

This is a classic form of hereditary bleeding. Inheritance occurs in a recessive type, sex-linked (X chromosome). Men who inherit the abnormal X chromosome from their mothers, who have no symptoms of the disease, get sick.

Clinic. Hemophilia can appear at any age. The earliest signs may be bleeding from a bandaged umbilical cord, hemorrhages in the skin, subcutaneous tissue in a newborn.

Bleeding in patients with hemophilia is prolonged, does not stop on its own, and is prone to relapse. Bleeding usually does not occur immediately after the injury, but after some time, sometimes after 6-12 hours or more. Any medical manipulation can lead to heavy bleeding. Tooth extraction and tonsillectomy lead to prolonged bleeding. After intramuscular injections, the occurrence of very extensive hematomas is typical.

Hemarthrosis (bleeding into the joints) is the most characteristic manifestation of hemophilia and the most common cause of disability in patients. Large joints are usually affected. At the first hemorrhages in the joint, the blood gradually resolves, and its function is restored. With repeated hemorrhages, the joint cavity is obliterated and loses its function - ankylosis develops.

Diagnosis based on the analysis of pedigree data (maternally male), anamnesis and laboratory data. Anemia, a significant prolongation of blood clotting time, a decrease in the amount of one of the blood coagulation factors are characteristic. Bleeding time has not changed.

Treatment consists in replacing the deficient factor and eliminating the consequences of hemorrhages. In local therapy, the application of tampons with a hemostatic sponge, thrombin, fibrin film to the site of bleeding, a defect in the mucous membranes and skin is used. In case of hemorrhage into the joint in the acute period, short-term immobilization of the limb in a physiological position for 2–3 days is indicated. For the treatment of hemarthrosis, phonophoresis with hydrocortisone, massage, and exercise therapy are used.

Hemophilia B. The process is based on a deficiency of IX coagulation factor. The path of inheritance and the clinical picture of the disease do not differ from hemophilia A.

Hemophilia C. The disease is associated with a deficiency of coagulation factor XI. The disease can be observed in both males and females. Clinically proceeds easily. It is usually characterized by minor subcutaneous hemorrhages, for which patients do not seek medical attention. Nosebleeds and hemarthroses are usually absent. Hemophilia C manifests itself during surgical interventions, extraction of teeth, injuries, when prolonged bleeding occurs, which is difficult to stop.

Hemophilia D associated with deficiency of XII coagulation factor. Clinically, the hemorrhagic syndrome is either completely unexpressed or slightly expressed. This form of the disease is called potential diathesis, i.e. diathesis, which can manifest itself under the influence of certain factors - surgical interventions, etc. The disease is observed in males and females. Blood clotting is normal or somewhat slow. As in hemophilia C, a decrease in the consumption of prothrombin and a weakening of thromboplastin generation in the Biggs-Douglas test are determined.

pseudohemophilia is a disease associated with a deficiency of factors involved in the II and III phases of blood coagulation. This group includes congenital and acquired forms of hypoprothrombinemia, deficiency of proaccelerin, proconvertin and fibrinogen. These forms of the disease are rare. Symptomatic pseudohemophilia can be observed with toxic liver damage. author Pariyskaya Tamara Vladimirovna

Chapter 8 Hemorrhagic diathesis Hemorrhagic diathesis is a group of diseases of various origins, which are united by the main clinical sign - increased bleeding. Bleeding can be the main symptom of the disease (for example, with hemophilia) or as

Hemorrhagic diathesis caused by changes in platelets Thrombocytopathies are the most common diseases in the group of hemorrhagic diathesis in children. Thrombopathies are based on a qualitative inferiority of platelets, usually with their normal number.