Hemorrhagic diathesis (lecture). Hemorrhagic diathesis Hemorrhagic diathesis

Definition 1

Hemorrhagic diathesis (HD) is a group of congenital and acquired diseases and syndromes, the main clinical manifestation which is increased bleeding.

Etiology and pathogenesis

Hemorrhagic diathesis, depending on the etiology, is divided into:

• Primary, arising as a result of hereditary deficiency of any blood coagulation factor.
• Symptomatic, which develop secondarily with various pathologies of cardio-vascular system, infectious diseases, tumors, overdose of certain drugs, etc.
• Neurotic: a group of rare HD that patients themselves cause as a result of mental disorders.

Remark 1

However, the immediate cause of clinical picture there may be a violation of the structure of the vascular wall (angiopathy), a decrease in the number of platelets in the blood or their defects (thrombocytopenia and thrombocytopathy), insufficiency of blood coagulation factors (coagulopathy), excessive fibrinolysis (hereditary or as a result of thrombolysis), DIC.

Clinical picture

Hemorrhagic diathesis got its name in connection with skin manifestations bleeding. Patients have hemorrhages under the skin and mucous membranes, frequent nasal, uterine, and other bleeding, hematuria and blood in the stool, vomiting of blood. Often, patients with minimal injuries experience bleeding into the joint cavity, which leads to the development of hemarthrosis.

There are several types of bleeding:

• Hematoma (with hereditary coagulopathy): massive hemorrhages predominate in soft tissues and joints spontaneous or post-traumatic.
• Spotty-petechial (with thrombocytopenia and thrombocytopathies): there are mainly superficial hemorrhages in the skin and mucous membranes, uterine bleeding with minor trauma.
• mixed microcirculatory-hematoma (with DIC, an overdose of anticoagulants): a combination of the above symptoms is characterized.
• vasculitic-purple (with hemorrhagic vasculitis): there is a combination of a hemorrhagic rash on the skin with inflammatory and immunoallergic phenomena.
• Angiomatous (with telangiectasias): characterized by persistent bleeding in the same localization.

Secondarily, as a result of regular blood loss, an anemic syndrome develops, manifested by general weakness, increased fatigue, dizziness, tinnitus, shortness of breath during exercise and palpitations, etc.

Diagnostics

In the diagnosis of these conditions, attention should be paid to the patient's family history and anamnesis of life, since the presence of chronic diseases the patient either hereditary pathologies circulatory systems in his relatives may indicate the cause of HD.

In the general blood test for HD, a decrease in the number of erythrocytes and hemoglobin content are determined. The color index can remain normal with a preserved ratio of hemoglobin and erythrocytes. It is necessary to estimate the number of platelets in the blood, which can be changed in any direction.

The most revealing study is a blood coagulogram, which will help to establish a deficiency of blood clotting factors.

Vascular or platelet damage can be assessed using spike or tourniquet tests. In the place of compression in this case, subcutaneous hemorrhages occur. Biochemical analysis blood can help in identifying underlying diseases.

In some cases, a bone marrow puncture is indicated to assess hematopoiesis.

Treatment

Therapy for HD depends on its etiology. These can be preparations of coagulation factors in case of their insufficiency, taking glucocorticoids in case of platelet pathology, and finally ascorutin and other vitamins in case of weakness of the vascular wall. An important method of treatment is the transfusion of blood components:

• platelet mass;
• fresh frozen plasma (restores the content of blood coagulation factors);
• erythrocyte mass (with severe blood loss).

Hemorrhagic diathesis

Hemorrhagic diathesis is a group of diseases, the main clinical sign which is a tendency to re-bleed or bleed, occurring both spontaneously and under the influence of minor injuries.

Etiology and pathogenesis of hemorrhagic diathesis. Stopping bleeding from damaged vessels and preventing spontaneous hemorrhages is provided by a complex of mechanisms called the hemostatic system.

Mechanisms of hemostasis:

1. Passive compression of the damaged vessel by blood that has poured into the perivascular space.

2. Reflex spasm of the damaged vessel.

3. Blockage of the damaged area of ​​the vascular wall by a thrombus of adherent platelets.

4. Reduction of the damaged vessel under the influence of serotonin, adrenaline, norepinephrine and similar substances released from the destroyed platelets.

5. Blockage of the damaged area of ​​the vascular wall with a fibrin thrombus.

6. Organization of a thrombus by connective tissue.

7. Scarring of the wall of the damaged blood vessel.

Classification of diathesis:

1) quantitative or qualitative insufficiency of platelets - thrombocytopenia and thrombocytopathy.

Thrombocytopenia is a group of diseases or syndromes, hereditary and acquired, in which the number of platelets in the blood is below 150 10 9 / l, which may be due to their increased destruction (the most common cause of these conditions) or insufficient formation.

Thrombocytopathies are violations of hemostasis caused by qualitative inferiority and dysfunction of blood platelets, occurring with a mildly reduced or normal content of platelets.

2) violations of coagulation hemostasis.

Among them, hereditary hemorrhagic coagulopathy, caused by a deficiency or molecular abnormalities of plasma coagulation factors, is primarily distinguished. The most common disease in this group is hemophilia A, associated with deficiency of factor VIII (antihemophilic globulin) and caused by X-linked recessive inheritance.

Acquired hemorrhagic coagulopathy is rarely caused by isolated deficiencies of individual coagulation factors alone. In many cases, they are strictly "tied" to certain clinical situations: infectious diseases, injuries, diseases. internal organs, renal and hepatic insufficiency, blood diseases, malignant neoplasms, drug (non-immune and immune) influences.

This group of coagulopathy includes the most common and potentially dangerous type of hemostasis pathology - disseminated intravascular coagulation syndrome (synonyms - DIC, thrombohemorrhagic syndrome). It is based on diffuse coagulation of circulating blood with the formation of many microclots and aggregates of blood cells that block blood circulation in the organs and cause deep dystrophic changes in them, followed by hypocoagulation, thrombocytopenia and hemorrhages. The syndrome has a diverse prevalence and rate of development - from lightning-fast lethal forms to latent and protracted ones, from general blood coagulation in the bloodstream to regional and organ thrombohemorrhages.

3) disorders of hemostasis of vascular and mixed genesis.

Damage to blood vessels, primarily capillaries, by various pathological processes can lead to the development of hemorrhagic syndrome in the absence of violations of the functional activity of the platelet and coagulation systems. The nature of hemorrhagic vasopathies can be allergic, infectious, intoxication, hypovitaminosis, neurogenic, endocrine and other.

In allergic vasopathies, there is a destruction of the components of the vascular wall containing autoallergens with autoantibodies and immunocytes, as well as the effect of allergen-antibody complexes and mediators of allergic reactions on it. Infectious and intoxicating vasopathies are the result of damage by infectious agents and toxins. Hypovitaminosis (C and P), neurogenic, endocrine vasopathies occur due to metabolic disorders in the vessel wall.

Clinical manifestations of hemorrhagic diathesis characterized by the five most common types of bleeding.

1. hematoma type, which occurs with a pronounced pathology of the blood coagulation system, is manifested by massive, deep, intense and painful hemorrhages into soft tissues, including muscles, subcutaneous and retroperitoneal tissue, into the peritoneum (abdominal catastrophes are simulated - appendicitis, peritonitis, intestinal obstruction), into the joints with their deformation, damage to cartilaginous and bone tissues and impaired function.

2. Petechial-spotted (bluish) type It is characterized by small painless punctate or spotted hemorrhages, not tense and not exfoliating tissues, which are provoked by injury to microvessels (rubbing of clothes, washing in a bath, light bruises, rubber bands from stockings). This type of bleeding accompanies thrombocytopenia and thrombocytopathy.

3. Mixed (bruising-hematoma) type characterized by a combination of signs of the two described types of hemorrhagic syndrome, often occurs in secondary hemorrhagic diathesis associated with disseminated intravascular coagulation, liver damage, overdose of anticoagulants and fibrinolytics.

4. Vasculitio-purple type, characterized by hemorrhages in the form of a rash or erythema, due to inflammatory changes in microvessels and perivascular tissue (immune vascular lesions, infections). Hemorrhages occur against the background of local exudative-inflammatory changes, and therefore the elements of the rash slightly rise above the level of the skin, are compacted, often surrounded by a rim of pigmented infiltration, and in some cases necrotic and crusted.

5. Angiomatous type occurs with vascular dysplasia (telangiectasia and microangiomatosis) and is characterized by persistent, repetitive bleeding from dysplastic vessels. The most frequent, profuse and dangerous nosebleeds.

Most often in therapeutic practice, hemorrhagic diathesis occurs due to a decrease in the content of platelets in the blood and damage to the vascular wall.

Thrombocytopeic purpura

Thrombocytopenic purpura (Werlhof's disease) is a hemorrhagic diathesis caused by a decrease in the number of platelets in the blood. There are 11 patients with this disease per 100,000 thousand of the population, and women suffer almost twice as often. The concept of "purpura" refers to capillary hemorrhages, petechial hemorrhages or bruises. Signs of bleeding occur when the platelet count falls below 150 10 9 /l.

Etiology. It is customary to distinguish hereditary and acquired forms of thrombocytopenic purpura. The latter arise as a result of immuno-allergic reactions, radiation exposure, toxic effects, including drugs.

Pathogenesis. The main element of the pathogenesis of thrombocytopenic purpura is a sharp shortening of the life span of platelets - up to several hours instead of 7-10 days. In most cases, the number of platelets formed per unit of time increases significantly (2-6 times compared to the norm). An increase in the number of megakaryocytes and overproduction of platelets are associated with an increase in the number of thrombopoietins in response to a decrease in the number of platelets.

In hereditary forms of the disease, the shortening of the life span of platelets is due to a defect in the structure of their membrane or a violation of the activity of glycolysis enzymes or the Krebs cycle. In immune thrombocytopenia, the destruction of platelets is a consequence of exposure to antibodies.

clinical picture. The first manifestations of the disease in most cases are acute, but subsequently it develops slowly and has a recurrent or protracted character.

Patients are concerned about the appearance on the skin and mucous membranes of multiple rashes in the form of small-point hemorrhages and bruises that occur spontaneously or under the influence of light bruises, pressure. At the same time, some hemorrhages disappear, but new ones appear. Often there is increased bleeding of the gums, nosebleeds. Women experience prolonged uterine bleeding.

On examination, the skin reveals hemorrhagic patches of purple, cherry blue, brown, and yellow flowers. They are noted mainly on the front surface of the body, in places of pressure on the skin of the belt, braces, garters. Often you can see hemorrhages on the face, conjunctiva, lips, at injection sites. Petechial lesions usually occur on the anterior surface of the legs.

When examining the cardiovascular, respiratory and digestive systems, changes characteristic of thrombocytopenic purpura are not observed.

Additional research methods.in peripheral blood sometimes with intense blood loss, posthemorrhagic anemia and an increase in the number of reticulocytes are observed. Main diagnostic

Hemorrhagic vasculitis

Hemorrhagic vasculitis (hemorrhagic immune microthrombovasculitis, Henoch-Schonlein disease) is an immunocomplex disease, which is based on multiple microthrombovasculitis that affects the vessels of the skin and internal organs.

Pathogenesis. The disease is characterized by aseptic inflammation of microvessels with more or less deep destruction of the walls, the formation of circulating low molecular weight immune complexes and activated components of the complement system. These phenomena cause microthrombovasculitis with fibrinoid necrosis, perivascular edema, blockade of microcirculation, hemorrhages, deep dystrophic changes up to necrosis.

clinical picture. The disease is manifested by the presence of skin, articular, abdominal syndromes associated with hemorrhages in the relevant areas, and a renal syndrome that develops as acute or chronic glomerulonephritis. Most often in clinical practice there is a skin-articular form of hemorrhagic vasculitis.

Patients complain about the occurrence of hemorrhagic rashes on the skin of the extremities, buttocks and trunk, the appearance of pain of varying intensity in large joints (often ankle, knee). Usually,. these pains occur simultaneously with the appearance of rashes on the skin. The debut of the disease is often accompanied by urticaria and other allergic manifestations.

In some cases, especially in young people, abdominal pain occurs, often severe, constant or cramping, usually disappearing on its own in 2-3 days.

On physical examination, the presence of small-dotted red, sometimes merging hemorrhagic rashes on the skin of the extremities and buttocks is determined. torso. Usually they are raised above the skin surface, located symmetrically mainly on the extensor surfaces of the lower extremities and around large joints. Often in these places there is pigmentation of the skin. When examining the joints, there is a limitation of their mobility, swelling of the periarticular tissues.

When examining the cardiovascular and respiratory systems, significant pathological changes, as a rule, are not observed.

Examination of the digestive system in the presence of abdominal syndrome can reveal bloating, pain on palpation of its various departments, tension in the abdominal wall.

Additional research methods. ATperipheral blood observed neutrophilic leukocytosis, increased ESR. The number of platelets is not changed. On biochemical examination, there may be an increase in the level α 2 - and β -blood globulins, fibrinogen, increase in circulating immunocomplexes.

Urinary syndrome is characterized by proteinuria (sometimes massive), micro- or macrohematuria, cylindruria.

Pinch and tourniquet symptoms in hemorrhagic vasculitis are usually positive. The duration of bleeding and the time of blood clotting do not change significantly.

Diagnostic criteria. The diagnosis of the disease is based on the presence of characteristic hemorrhagic rashes of the vasculitic-purple type, arthralgia, abdominal and renal syndromes, increased capillary fragility (positive pinch and tourniquet tests), and the absence of pronounced changes in the hemostasis system.

Formulation of a detailed clinical diagnosis.Example. hemorrhagic vasculitis, chronic course, skin-articular form.

symptom is thrombocytopenia. Typically, thrombocytopenic purpura occurs when the platelet count falls below 50-10%. An increase in the size of platelets is often found; their poikilocytosis, the appearance of small-grained "blue" cells. Often there are violations of the functional activity of platelets in the form of a decrease in their adhesion and aggregation. AT puiktap|e bone marrow most patients have an increased number of megakaryocytes, which are no different from normal ones. Only with an exacerbation of the disease, their number temporarily decreases. In platelets and megakaryocytes, the content of glycogen is reduced, the ratio of enzymes is disturbed.

Significant importance in the diagnosis of hemorrhagic diathesis belongs to the study state of hemostasis. l

Approximately increased capillary fragility is judged by a positive pinch test - the formation of a bruise during compression of the skin fold in the subclavian region. More precisely, the resistance of capillaries is determined using a tourniquet test, based on the appearance of petechiae below the place where the cuff of the measuring device is applied to the upper arm.

blood pressure when creating a pressure of 90-100 mm Hg in it. Art. After 5 minutes inside a circle with a diameter 5 cm, previously outlined ~ 1 on the forearm, the number of petechiae with a weakly positive sample can reach 20 (the norm is up to 10 petechiae), with a positive one - 30, and with a sharply positive one and more.

The duration of bleeding is determined by piercing the skin at the lower edge of the earlobe with a depth of 3.5 mm. Normally, it does not exceed 4 minutes (Duke's test).

The state of the internal mechanism of blood coagulation can be judged directly at the patient's bedside using the Lee-White method: 1 ml of blood collected in a dry test tube normally coagulates after 7-11 minutes.

With thrombocytopenic purpura, positive symptoms of a pinch and tourniquet are noted. The duration of bleeding is significantly lengthened (up to 15-20 minutes or more). Blood clotting in most patients is not changed.

Diagnostic criteria. The diagnosis of thrombocytopenic purpura is based on the presence of a characteristic clinical picture, a petechial-spotted type of hemorrhage in combination with nasal and uterine bleeding, severe thrombocytopenia, increased fragility of capillaries and an increase in the duration of bleeding.

Formulation of a detailed clinical diagnosis. Example. 7 rhombocytopenic

ia purpura, relapsing form, physical exacerbations.

1. Hemorrhagic diathesis caused by a violation of thrombocytopoiesis or platelet hemostasis (thrombocytopathy).

• Thrombocytopenic purpura (idiopathic and acquired).
• Symptomatic thrombocytopenia (leukemia, hemorrhagic aleukia, radiation sickness, etc.).
• Thrombocytopathies (violations of aggregation-adhesive and other functions of platelets).
• Hemorrhagic thrombocythemia.

1. Hemorrhagic diathesis caused by impaired blood clotting and fibrinolysis or coagulation hemostasis (coagulopathy).
2. Violation of thromboplastin formation, or the 1st phase of blood coagulation.

• Hemophilia A, B and C.

1. Violation of thrombin formation, or the 2nd phase of blood coagulation (dysprothrombia).

• Hypoproaccelerinemia (parahemophilia).
• Hypoproconvertinemia.
• Factor X deficiency (Stuart-Prower).

Hypoprothrombinemia (hemorrhagic diathesis of newborns; endogenous K-avitaminosis with obstructive jaundice; liver damage; drug-induced or dicoumarium hemorrhagic diathesis after an overdose of indirect anticoagulants). Violation of the formation of thrombin (drug hemorrhagic diathesis after an overdose of direct anticoagulants such as heparin).

1. Violation of fibrin formation, or the 3rd phase of blood coagulation.

Afibrinogenemic purpura (congenital). Fibrinogenopathy (acquired hypofibrinogenemia). Deficiency of fibrin-stabilizing (XIII) factor.

1. fibrinolysis disorder.

Fibrinolytic bleeding and hemorrhage caused by acute fibrinolysis due to thrombohemorrhagic syndrome (disseminated intravascular coagulation syndrome, consumption coagulopathy) and an overdose of thrombolytic drugs.

1. Violation of blood coagulation in various phases due to circulating anticoagulants (antithromboplastins, inhibitors of factors VIII and IX, antithrombins).

III. Hemorrhagic diathesis caused by damage to the vascular wall (vasopathy).

Hemorrhagic vasculitis (Schonlein-Genoch disease). Hemorrhagic purpura associated with infectious-toxic, infectious-allergic, dystrophic and neuroendocrine effects.

Hemorrhagic angiomatosis (Rendu-Osler-Weber disease), C-avitaminosis (scorbut).

According to 3.C. Barkagan, with hemorrhagic diathesis, the following main types of bleeding should be distinguished:

1. Hematoma. It is characteristic for disorders of the internal mechanism of blood coagulation - hereditary (hemophilia) and acquired (appearance of circulating anticoagulants in the blood). Sometimes observed with an overdose of anticoagulants (retroperitoneal hematomas).
2. Capillary, or microcirculatory. Characteristic for thrombocytopenia and thrombocytopathy, as well as deficiency of plasma factors of the prothrombin complex (V, VII, X, II), hypo- and dysfibrinogenemia; manifested by petechial-spotted hemorrhages in the skin, mucous membranes, bleeding from the gums, uterine, nasal.
3. Mixed capillary hematoma. Characteristic for disseminated intravascular coagulation (thrombotic hemorrhagic syndrome), von Willebrand disease (deficiency of factor VIII, vascular factor and violation of the adhesive-aggregative function of platelets), overdose of anticoagulants. It is manifested mainly by hematomas and petechial-spotted hemorrhages.
4. Purple. It is observed in hemorrhagic vasculitis and other endotheliosis. It is manifested mainly by symmetrically located small dotted and erythemal hemorrhages.
5. Microangiomatous. It is caused by hereditary and acquired vascular dysplasias (Randu-Osler disease, symptomatic capillaropathy). It is characterized by persistent repetitive bleeding of the same localization.

Not all of the hemorrhagic diatheses listed above can be attributed to emergency conditions, however, in many of them, at certain periods, the hemorrhagic syndrome is so pronounced that emergency therapy is necessary.

These most common hemorrhagic diatheses in the therapeutic clinic include thrombocytopenic purpura (a type of thrombocytopathy), hemophilia, drug-induced (dicumarin and heparin) hemorrhagic diathesis, fibrinolytic bleeding and hemorrhage in thrombohemorrhagic syndrome (multiple intravascular coagulation syndrome) and overdose of streptokinase (representatives of coagulopathy) , hemorrhagic vasculitis and hemorrhagic angiomatosis (types of vasopathy).

The reasons

There are hereditary (family) forms with a long-term, starting with childhood bleeding and acquired forms are mostly secondary (symptomatic). Most of the hereditary forms are associated with abnormalities of megakaryocytes and platelets, dysfunction of the latter, or with a deficiency or defect in plasma coagulation factors, as well as von Willebrand factor, less often with inferiority of small blood vessels.

Most acquired forms of bleeding are associated with DIC syndrome, immune and immunocomplex lesions of the vascular wall (Schonlein-Genoch vasculitis, erythema, etc.) and platelets (most thrombocytopenia), with impaired normal hematopoiesis (hemorrhages in leukemia, hypo- and aplastic states of hematopoiesis, radiation sickness), toxic-infectious lesions of the blood vessels ( hemorrhagic fevers, typhus etc.), liver diseases and obstructive jaundice (leading to impaired synthesis of blood coagulation factors in hepatocytes), exposure to drugs that disrupt hemostasis (deaggregants, anticoagulants, fibrinolytics) or provoke immune disorders - thrombocytopenia, vasculitis.

In many of the listed diseases, hemostasis disorders are of a mixed nature and sharply increase due to secondary development. DIC syndrome, most often in connection with infectious-septic, immune, destructive or tumor (including leukemia) processes.

Symptoms

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. Nasal and gingival bleeding, hemorrhages in the mucous membranes, 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 diseases 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.

Manifestations of hemorrhagic diathesis in children

The main manifestation of any hemorrhagic diathesis is increased bleeding. Depending on the causes of occurrence, 5 of its variants are distinguished:

• Hematoma. With this type of bleeding, any blow or fall leads to an outpouring of blood in large quantities into soft tissues, joints, internal cavities, and a violation of the integrity skin or mucous provokes the development of severe bleeding. This type of bleeding is characteristic of hemophilia and acquired coagulopathy (blood does not clot for a very long time, so heavy bleeding occurs).
• capillary(it is also called microcirculatory). The main manifestations are small blood spots (petechiae and ecchymosis) on the skin and mucous membranes. There may be oozing nosebleeds, bleeding gums. This type of bleeding occurs mainly with thrombocytopenia and thrombocytopathies.
• mixed. In this condition, there are signs of the two previous forms of bleeding, that is, hematomas and small blood spots. There is a condition when there are violations of both the platelet system and the plasma coagulation factors. For example, a similar picture is very characteristic of von Willebrand's disease.
• Angiomatous. By name, it is clear that this form of bleeding develops due to the pathology of the walls of blood vessels. The main manifestations are persistent bleeding from the nose, the digestive tract (usually from the same vessels).
• Vasculitic purple. The main problem and cause of this variant of bleeding is immune, toxic or allergic damage to small vessels. Hemorrhages appear most often symmetrically in the area of ​​large joints, although intense internal bleeding is also possible. This type of bleeding is observed in Henoch-Schonlein disease.

All other symptoms of hemorrhagic diathesis in most cases are the result of hemorrhagic syndrome. Children may have pain in the joints (due to the formation of hematomas in them), the stomach (with bleeding in the gastrointestinal tract), signs of anemia (pallor, weakness, dizziness) may appear. If the kidney vessels are affected, the color of the urine may change. Neurological disorders may also be present - this is a sign of hemorrhages in the brain. In general, the condition of a child suffering from hemorrhagic diathesis worsens significantly every day.

When to see a doctor

Parents should periodically examine children for bruising. Normally, children may have several bruises on their shins, since the lower limbs are a very vulnerable place, especially for babies who have begun to walk actively. In addition, bruises of a certain localization may be in children involved in sports (for example, football, wrestling). If there are no other complaints other than a few bruises, there is no need to panic.

But sound the alarm and turn to pediatrician required in the following cases:

• If hematomas form for no apparent reason and in unusual places (arms and legs are usually common places), for example, on the back, chest, stomach, face.
• If, after minor damage to soft tissues, prolonged bleeding occurs.
• If there is blood in the stool or urine.
• If the child's skin has become pale.
• If the child has increased fatigue.

Diagnosis of hemorrhagic diathesis

The general diagnosis of hemorrhagic diseases and syndromes is based on the following main criteria:

• determination of the timing of onset, prescription, duration and characteristics of the course of the disease (appearance in early childhood, adolescence, or in adults and the elderly, acute or gradual development of hemorrhagic syndrome, recent or long-term (chronic, recurrent) course, etc .;
• identification, if possible, of a family (hereditary) genesis of bleeding (with specification of the type of inheritance) or an acquired nature of the disease; clarification of the possible connection between the development of hemorrhagic syndrome and previous pathological processes, influences (including therapeutic ones - medications, vaccinations, etc.) and background diseases (liver disease, leukemia, infectious-septic processes, injuries, shock, etc.);
• determination of the predominant localization, severity and type of bleeding. So, with Osler-Randu disease, persistent nosebleeds predominate and are often the only ones; with platelet pathology - bruising, uterine and nosebleeds, with hemophilia - deep hematomas and hemorrhages in the joints.

A plan for examining a patient with increased bleeding syndrome is drawn up by a hematologist together with a treating specialist (rheumatologist, surgeon, obstetrician-gynecologist, traumatologist, infectious disease specialist, etc.).

First of all, research clinical tests blood and urine, platelet count, coagulogram, feces occult blood. Depending on the results obtained and the proposed diagnosis, an extended laboratory and instrumental diagnostics(biochemical blood test, sternal puncture, trepanobiopsy). In hemorrhagic diathesis of immune origin, the determination of anti-erythrocyte antibodies (Coombs test), anti-platelet antibodies, lupus anticoagulant, etc. is shown. Additional Methods may include functional tests for fragility of capillaries (tests of tourniquet, pinch, cuff test, etc.), ultrasound of the kidneys, ultrasound of the liver; radiography of the joints, etc. To confirm the hereditary nature of hemorrhagic diathesis, a consultation with a geneticist is recommended.

Treatment of hemorrhagic diathesis

Before starting treatment, it is important to successfully diagnose.

This may require the following:

• Conducting general laboratory and biochemical research blood and urine.
• Determination of the time required for blood clotting.
• Conducting immunological, as well as a test for the generation of thromboplastin, prothrombin and thrombin tests.
• Laboratory examination of blood serum.
• Carrying out a coagulogram.

After passing these examinations, the doctor can make an accurate diagnosis and prescribe the appropriate treatment. It may include taking certain medications (eg, iron supplements, corticosteroids) and vitamin complexes and supplements, or more radical methods: for example, puncture of a joint, transfusion of plasma or red blood cells, or surgical removal of the spleen.

When diagnosing hemorrhagic diathesis, it is important to take preventive measures that can strengthen the body and increase its protective functions. These include hardening, moderate physical exercise and exercise, a balanced diet with a carefully selected vitamin composition. In addition, it is especially important to protect the body from the possibility of infection. viral diseases during the autumn-spring period. For this, it is recommended to take specially selected vitamin complexes.

When choosing a treatment, a differentiated approach is practiced, taking into account the pathogenetic form of hemorrhagic diathesis. So, with increased bleeding caused by an overdose of anticoagulants and thrombolytics, the abolition of these drugs or correction of their dose is indicated; the appointment of vitamin K preparations (vikasol), aminocaproic acid; plasma transfusion. Therapy of autoimmune hemorrhagic diathesis is based on the use of glucocorticoids, immunosuppressants, plasmapheresis; with an unstable effect from their use, a splenectomy is required.

In case of hereditary deficiency of one or another coagulation factor, it is indicated to carry out replacement therapy their concentrates, transfusions of fresh frozen plasma, erythrocyte mass, hemostatic therapy. In order to locally stop small bleeding, the application of a tourniquet, pressure bandage, hemostatic sponge, ice is practiced; conducting nasal tamponade, etc. With hemarthrosis, therapeutic punctures of the joints are performed; with hematomas of soft tissues - their drainage and removal of accumulated blood.

The main principles of the treatment of DIC include the active elimination of the cause given state; cessation of intravascular coagulation, suppression of hyperfibrinolysis, replacement hemocomponent therapy, etc.

Complications and prognosis of hemorrhagic diathesis

Complications hemorrhagic diathesis dependent on the location of the hemorrhage. With repeated hemorrhages in the joints, hemarthroses occur with the formation of extensive hematomas in the area of ​​​​the passage of large nerve trunks, compression of the nerves with the development of paralysis, paresis, with hemorrhages in the brain, symptoms appear characteristic of cerebrovascular accident.

With repeated transfusions of blood and plasma, serum hepatitis may develop, in patients with total absence coagulation factors, the formation of antibodies is possible, which significantly reduces the effectiveness of transfusions; possible post-transfusion reactions.

Blood diseases are often diagnosed today. Among them there are complex pathologies circulatory system, which lead to a violation of blood clotting, as a result of which people develop hemorrhagic diathesis. This disease is characterized by sudden progressive bleeding and hemorrhage, varying in duration and severity. Such phenomena can be observed in the form of small rashes, large hematomas and even internal bleeding. A sick person then develops an anemic syndrome. In the absence of therapy, the development of serious pathologies that can lead to death is possible.

Description of the problem

Hemorrhagic diathesis is a disease of the circulatory system, which is characterized by the body's tendency to spontaneous bleeding and hemorrhage as a result of the presence of defects in one or more blood coagulation mechanisms.

Medicine knows about three hundred varieties of these pathologies. According to statistics, five million people in the world suffer from this disease. In some cases, the affected area is very large, so a person often has complications of the disease. Because of all these factors, the problem of the disease is under control in medical professionals such as surgeons, hematologists, traumatologists, gynecologists and obstetricians.

Types of disease

It is customary to distinguish two types of disease:

1. Congenital hemorrhagic diathesis in children is usually inherited. This pathology accompanies a person from birth to the end of life. This form of diathesis is complicated by hemophilia, Glanzman's syndrome, telangiectasia, thrombocytopathy and other pathologies. In this case, there is a lack of one or more blood elements that ensure its coagulability.
2. Acquired hemorrhagic diathesis in children and adults develops as a result of inflammatory process or blood disorders. Such pathologies include vasculitis, liver disease with vascular damage, intoxication of the body with medications, infectious diseases, purpura and others. Acquired diathesis can be symptomatic, which appears against the background of pathologies of the cardiovascular system or cancerous neoplasms, and neurotic, which develops against the background of mental disorders.

Also, diathesis can be primary, developing as an independent disease, and secondary, appearing as a result of previously transferred diseases of an infectious nature, poisoning or sepsis.

Hemorrhagic diathesis. Classification

In medicine, several groups of pathologies are distinguished depending on the disorder of one of the hemostasis factors:

1. Thrombocytopenia, as well as thrombocytopathies, which are characterized by a violation of platelet hemostasis. This phenomenon can be observed in the disorder immune system, radiation sickness, leukemia, purpura and so on.
2. Coagulopathy, which is caused by a disorder of coagulation hemostasis, which contributes to the final stop of blood. These phenomena are observed in the development of diseases such as hemophilia, Stuart-Prauer or Willebrand syndrome, fibrinogenopathy and others. The disease can be formed due to the use of anticoagulants and fibrinolytics.
3. Vasopathies that manifest themselves in violation vascular walls. Such pathologies develop with vasculitis, beriberi, Rendu-Osler syndrome.
4. Mixed diathesis is characterized by disorders that are included in the first and second groups. This group includes von Willebrand syndrome, thrombohemorrhagic disease, hemoblastosis and others.

Types of bleeding

Types of bleeding in hemorrhagic diathesis are as follows:

1. The hematoma type is characterized by the presence of large hematomas, bleeding after surgical procedures, hemorrhages in the joints.
2. The capillary type is caused by the development of small hemorrhages in the form of petechiae and ecchymosis, nasal, gastric and uterine bleeding and bleeding gums. At the same time, bruises and small red rashes form on the human body.
3. The purple type is caused by the presence of small symmetrical rashes and spots on the skin.
4. Microangiomatous type, in which bleeding is periodically repeated due to impaired development of small vessels. Groups of burgundy spots appear on the skin, which do not tend to spread throughout the body.
5. mixed type, in which both hematomas and small spots form on the skin.

Reasons for the development of the disease

Hemorrhagic diathesis can occur at any age. The reason for this is a violation of blood clotting, a disorder in the functionality of platelets, an increase in the permeability of the walls of blood vessels. People with certain congenital and acquired pathologies are prone to this disease. Primary forms of the disease are the result of a hereditary predisposition and are associated with congenital anomalies or a lack of one of the hemostasis factors.

Congenital pathologies develop due to the following genetic diseases:

1. Hemophilia. Hemorrhagic diathesis in this case is formed due to congenital low level blood clotting. Most often, internal bleeding occurs.
2. Lack of prothrombin, which is characterized by a congenital disorder of its synthesis.
3. Thrombosthenia, Bernard-Soiler syndrome are characterized by a disorder in the functionality of platelets. The disease manifests itself on the skin.
4. Willebrand syndrome, which is caused by abnormal activity of a protein responsible for hemostasis.
5. Glanzman's disease, Randu - Osler, Stuart - Prouer.

Such pathologies are quite rare today.

Causes of secondary diathesis

Most often, diathesis develops as an acquired disease. Its appearance can provoke the following diseases:

1. Pathology of the liver and kidneys, cirrhosis.
2. Lack of vitamin K, in particular in infants, which leads to fragility and fragility of blood vessels.
3. Autoimmune diseases, as a result of which antibodies attack your own platelets.
4. Vasculitis, sepsis, shock, resulting in damage to blood vessels.
5. Exposure to high doses of radioactive radiation, exposure to chemotherapy contribute to the disorder of platelet formation.
6. Taking certain medications, such as steroids.
7. Mental illness, accompanied by self-harm.
8. Advanced age.

Symptoms and signs of pathology

Symptoms of hemorrhagic diathesis are often different, depending on the disease, the consequences of which they act. Signs of pathology are vivid. With the defeat of the vascular walls in a person throughout the body, small rash, including mucous membranes. In some cases, there is pain in the abdomen and joints, the presence of blood in the urine, swelling.

With the development of coagulopathy, the patient suddenly develops bleeding, extensive subcutaneous hemorrhages, which change the color of the skin. Then the person develops anemia. With hemophilia in children in the first year of life, nosebleeds and subcutaneous hemorrhages, arthralgia, swelling of the joints are observed.

In severe cases, diathesis leads to the appearance of ulcers, nausea and vomiting with blood, pain in the abdomen and lower back, enlargement of the liver and spleen, anemia, dysuria. With anemia, a person has weakness, hypotension, tachycardia, dizziness, pallor of the skin.

Signs of pathology in children and pregnant women

In children, hemorrhagic diathesis (photo presented) may be one of the signs acute leukemia. Pathology often manifests itself in gingival bleeding during teething, nosebleeds, skin rashes, joint pain and deformities, as well as retinal hemorrhages, vomiting and defecation mixed with blood. Adolescent girls experience heavy menstruation.

Often, thrombocytopathy in children is not a disease, it indicates the immaturity of platelets. This phenomenon disappears after puberty. But doctors recommend taking this phenomenon seriously, since often under the influence of negative factors internal bleeding occurs during injuries or a stroke.

In pregnant women, with this pathology, late toxicosis, dropsy, there is a threat of miscarriage, placental insufficiency, premature birth. In such women, children are often born prematurely, they have hypoxia, developmental delay.

Complications and consequences

Complications of this pathology are:

• chronic anemia;
• the occurrence of allergies, which also contribute to the development of bleeding;
• Hepatitis B;
• HIV infection in case of repeated blood transfusion;
• limitation or complete immobility of the joints;
• numbness and paralysis of tissues;
• intracranial hemorrhage, paresthesia, paresis or paralysis;
• blindness, stroke;
• coma.

These complications do not occur in all patients, they appear depending on the type of pathology and associated negative factors. At timely treatment development negative consequences manages to avoid. But with uncontrolled blood loss, complications will develop.

Diagnostic measures

When contacting a doctor, he studies the factors that could cause the development of the disease, the form and prevalence of the pathology.

Hemorrhagic diathesis is diagnosed using the following methods:

1. Laboratory tests of blood, feces and urine.
2. Analysis of blood serum to determine the content of microelements in the body.
3. Blood clotting test.
4. Coagulogram.
5. Thromboplastin and Coombs generation tests, thrombin and prothrombin analyses.
6. Immunological research.
7. Ultrasound of the kidneys and liver.
8. bone marrow research.
9. X-ray of the joints.

After a comprehensive examination, the doctor makes a final diagnosis and prescribes appropriate therapy.

Therapeutic measures

Treatment of hemorrhagic diathesis should be complex, consisting of the following steps:

1. Medical therapy, which is aimed at increasing the level of platelets, increasing blood clotting, strengthening the vascular walls. In this case, vitamins, aminocaproic acid, glucocorticosteroids, immunosuppressants are prescribed.
2. Stop bleeding. To do this, the doctor uses a tourniquet, a special sponge, cotton swabs, a heating pad with ice.
3. Surgery. Often, the spleen is extracted to increase the lifespan of blood cells, as well as to eliminate the pathological vessel, which is replaced by a prosthesis, a puncture of the joint cavity to eliminate blood.
4. Physiotherapy.
5. Blood transfusion to increase platelet and electrocyte levels.

After hemorrhagic diathesis is cured, clinical guidelines given by the attending physician. He usually prescribes a diet that involves the complete exclusion of fast food, preservatives, convenience foods and sauces. To increase the concentration of platelets, you can eat vegetables such as beets, broccoli, celery, tomatoes, and beef liver.

Forecast

The course of the disease and its prognosis may be different. With timely effective therapy the prognosis will be favorable. In the absence of treatment or the development of serious complications, a fatal outcome is possible. Therefore, it is important to diagnose the disease in a timely manner in order to reduce the risk of complications in the future.

Prevention

What should be done to identify hemorrhagic diathesis? Doctors' recommendations are unequivocal - testing for the presence of a genetic predisposition to pathology. It is also necessary to plan pregnancy, undergo the necessary tests and consultations, conduct healthy lifestyle life during childbearing. It is also important to eat right, regularly undergo scheduled examinations and examinations, give up addictions and uncontrolled use of medications, avoid injuries and injuries, monitor your health and the health of your children.

By following all the recommendations and prescriptions of a doctor, you can avoid serious and dangerous complications this disease. Since it is chronic, it is necessary to periodically be examined by specialists and take a course of therapy.