Hemorrhages - what is it? Causes and treatment of hemorrhage. Hemorrhagic diseases symptoms and signs

Hemorrhage is a pathology in which there is a release of blood from the lumen of a vessel or through vascular tissue into environment, or on the surface of the skin, into the external environment. The cause is damage to the vessel walls due to injury or without it (anomaly of platelets, megakaryocytes, blood plasma, blood coagulation system). The consequences of hemorrhage depend on its severity. Sometimes this doesn't lead to serious consequence, and sometimes can cause anemia and even lead to fatal outcome. If blood accumulates inside the body's tissues, it causes compression of the internal organs, which impairs their functionality. Since bleeding itself is a consequence of the disease, the cause itself must be treated. Accordingly, for such a phenomenon as hemorrhage, treatment will need to be prescribed by a specialist after a thorough examination.

Hemorrhages at the injection site

Hemorrhages at the injection site appear after individual drugs or due to hemorrhagic diseases. There is a method by which the permeability of the vessel wall can be determined: 1-2 ml of saline solution is injected into the skin, after which the manifestation of hemorrhages is observed. This method was called Hess's symptom (British scientist, 19th century).

Hemorrhages on the skin

Hemorrhages on the skin appear due to hemorrhages when blood enters the dermal tissue. Such hemorrhages can be of the following types:

Subcutaneous hematomas: hemorrhages in the tissue under the upper layer of the dermis. The resulting voids under the skin are filled with clotted blood. At the site of the hematoma, a swelling forms, changing color over time from bright red-blue to yellow-green;

Ecchymoses (bruises): have different sizes and appear due to subcutaneous hemorrhages;

Petechiae: capillary hemorrhages on the skin. These pinpoint hemorrhages range in size from a dot left by a gel pen to the size of a kidney bean.

Hemorrhages in the stomach

Bleeding in the stomach may be a consequence of hemorrhagic erosive gastritis. However, hemorrhages in the stomach are not the only symptom of the disease. The patient may experience erosive and inflammatory changes in the mucous membrane and high gastric secretion. Sometimes bleeding can be caused by high permeability of the walls of the stomach vessels, as well as high degree susceptibility to damage.

Expulsive hemorrhage

Expulsive hemorrhage - severe postoperative complication on eyeball represents bleeding from the ciliary arteries. Bleeding may begin during surgery. However, most often, rupture of the ciliary arteries occurs after surgery due to severe vasodilation due to reactive hypertension. It is observed in patients suffering from atherosclerosis, hypertension, and eye hypertension. Before surgery, these factors must be taken into account by doctors. Sometimes such bleeding can cause the choroid and retina to be pushed out through the surgical wound, leading to loss of vision. Therefore, expulsive hemorrhage requires urgent surgical intervention: posterior trepanation of the sclera, application of scleral sutures.

Intramucosal hemorrhages

Intramucosal hemorrhages are a tendency for blood vessels in the mucous membrane to bleed due to injury or disease. This syndrome can be acquired or congenital. In addition, this tendency can be inherited. Their main cause is considered to be a violation of one or more links of hemostasis.

Among the hereditary causes of hemorrhage, it is worth highlighting the most common diseases: hemophilia types A, B, von Willebrand disease, thrombocytopathy. The causes of acquired mucosal hemorrhagic syndrome can be: DIC syndrome, hemorrhagic vasculitis, thrombocytopenia, thrombocytopathy and deficiency of prothrombin complex factors.

Hemorrhagic diseases and syndromes are pathological conditions characterized by increased bleeding as a result of insufficiency of one or more elements of hemostasis.

Etiology

There are hereditary and acquired forms of hemorrhagic diseases and syndromes.

Hereditary forms are associated with genetically determined pathological changes in the vascular wall, anomalies of megakaryocytes, platelets, adhesion proteins of blood plasma and plasma factors of the blood coagulation system.

Acquired forms in most cases are caused by damage blood vessels immune, immunocomplex, toxic-infectious and dismetabolic etiology (various vasculitis), damage to megakaryocytes and platelets of various etiologies (thrombocytopathy), pathology of adhesion proteins of blood plasma and factors of the blood coagulation system and multifactorial disorders of the blood coagulation system ( acute syndromes ICE).

Kinds hemorrhagic diseases

By origin they distinguish the following types hemorrhagic diseases and syndromes: vasculitis, thrombocytopenia, thrombocytopathy, coagulopathy, disseminated intravascular coagulation.

Vasculitis. Caused by primary damage to the vascular wall with the possible secondary development of coagulation and platelet disorders. This group includes hereditary hemorrhagic telangiectasia Randu–Osler, syndrome Ehlers –Dunlo , syndrome Marfana , giant hemangiomas in the syndrome Kazabakh–Merritt, hemorrhagic vasculitis Schönlein–Henoch, erythema, hemorrhagic fevers, hypovitaminosis C and B, etc.

Thrombocytopenia. They develop as a result of primary damage to the megakaryocyte-platelet lineage, redistribution of platelets and their deposition in the spleen, increased destruction (for example, in SLE or idiopathic thrombocytopenic purpura), increased consumption of platelets and the formation of blood clots (DIC, thrombotic thrombocytopenic purpura), and the use of certain drugs.

Thrombocytopathies. Characterized by the presence of abnormal platelets with disruption of their functions. The most common among them is thrombasthenia Glyantsmann and illness background von Willebrand .

Coagulopathies. Caused by blood clotting disorders.

† Hereditary coagulopathies: hemophilia A, hemophilia B, disease background von Willebrand , deficiency of blood clotting factors;

† Acquired coagulopathies: vitamin K-dependent coagulopathies (occur with insufficient liver function, impaired absorption of vitamin K, nutritional deficiency of vitamin K, taking drugs such as coumarin), disseminated intravascular coagulation, liver pathology (leads to a deficiency of many coagulation factors), pathological coagulation inhibitors ( lupus anticoagulant; specific coagulation inhibitors - ATs specific to individual coagulation proteins);

† Impaired fibrin stabilization, increased fibrinolysis, including during treatment with direct and indirect anticoagulants, fibrinolytics (streptokinase, urokinase, alteplase, etc.);

† Other acquired coagulation disorders: Deficiency of clotting factors can occur with somatic diseases (for example, with amyloidosis - factor X deficiency).

ICE. They are a consequence of complex disorders of various parts of the hemostasis system.

Types bleeding

The following types of bleeding are distinguished.

Capillary or microcirculatory (petechial-bruise) type of bleeding. It is characterized by petechial rashes, bruises and ecchymoses on the skin and mucous membranes. Often combined with increased bleeding of the mucous membranes (nosebleeds, menorrhagia). Severe hemorrhages in the brain may develop. This type of bleeding is characteristic of thrombocytopenia and thrombocytopathies, diseases background von Willebrand , insufficiency of prothrombin complex factors (VII, X, V and II), some variants of hypo- and dysfibrinogenemia, moderate overdose of anticoagulants.

Hematoma type of bleeding. Characterized by painful, intense hemorrhages in subcutaneous tissue, muscles, large joints, into the peritoneum and retroperitoneal space. Hematomas can lead to compression of nerves, destruction of cartilage and bone tissue, dysfunction of the musculoskeletal system. Sometimes renal and gastrointestinal bleeding develop. Prolonged bleeding is typical from cuts, wounds, after tooth extraction and surgical interventions, often leading to the development of anemia. This type of bleeding is observed in some hereditary bleeding disorders (hemophilia A and B, severe deficiency of factor VII), acquired coagulopathies, accompanied by the appearance of inhibitors of factors VIII, IX, VIII + V in the blood, and with an overdose of anticoagulants, as well as in hereditary thrombocytopathy with absence of lamellar factor 3.

Mixed capillary-hematoma type of bleeding. It is characterized by petechial bruises, combined with extensive dense hemorrhages and hematomas. Observed in hereditary (severe deficiency of factors VII and XIII, severe form of the disease background von Willebrand ) and acquired (acute DIC syndromes, significant overdose of direct and indirect anticoagulants) disorders.

Vasculitic purpuric type of bleeding. Manifests as hemorrhagic or erythematous (on inflammatory basis) rashes, possible development of nephritis and intestinal bleeding; observed in infectious and immune vasculitis.

Angiomatous type of bleeding. It is characterized by repeated strictly localized bleeding associated with local vascular pathology. Observed for telangiectasis, angiomas, arteriovenous shunts.

Basic causes bleeding

The main causes of hypocoagulation and bleeding are presented in Fig. 21–27.

Rice. 21–27. The main causes of hypocoagulation of blood proteins and hemorrhagic syndrome.

Mechanisms Hypocoagulation

The mechanisms of hypocoagulation and bleeding are presented in Fig. 21–28.

Rice. 21–28. Basic mechanisms of blood hypocoagulation and hemorrhagic syndrome.

Hemorrhagic diseases and syndromes can be caused by pathologies of blood vessels (vasopathies), platelets (thrombocytopathies), and the hemostatic system (coagulopathies).

Hemorrhagic diseases, conditional pathology vessels

Typical diseases of this group are Randu–Osler, purple Schönlein–Henoch, primary hemorrhagic vasculitis.

Disease Randu–Osler

Randu–Osler–Weber disease (hereditary hemorrhagic telangiectasia, hereditary hemorrhagic angioma, disease Osler–Weber, disease Osler) - hereditary () angiopathy, manifested by multiple telangiectasia and hemorrhagic syndrome. Frequency. 1:16,000 population.

Treatment and prevention

To stop bleeding, local and general hemostatic therapy is used (irrigation with thrombin solution and 5% aminocaproic acid solution, nasal tamponade with oil swabs, detachment of the mucous membrane in the area of ​​bleeding, cauterization). Cryotherapy is more effective. Sometimes it is necessary to resort to surgical treatment (excision of angiomas, plastic surgery of the nasal septum, ligation and embolization of arteries). Barotherapy and laser cauterization are also used. With concomitant factor deficiency background von Willebrand Transfusions of fresh frozen plasma and administration of cryoprecipitate are carried out. If anemia develops, blood transfusions are performed and iron supplements are prescribed.

Patients should avoid traumatizing the mucous membranes at the sites of angiomas. The nasal mucosa is lubricated with lanolin (with thrombin) or neutral oils. When getting married, medical genetic counseling is necessary.

Hemorrhagic vasculitis

Hemorrhagic vasculitis (anaphylactoid purpura, immune complex vasculitis, disease Schönlein–Henoch) - bleeding caused by damage to small vessels by immune complexes and components of the complement system (see the article “Purpura” in the Appendix “Reference of Terms” on the CD).

Hemorrhagic syndromes, conditional pathology platelets

Hemorrhagic syndromes caused by platelet pathology (thrombocytopenia and thrombocytopathy) include idiopathic thrombocytopenic purpura, thrombasthenia Glyantsmann , syndrome Bernard –Soulier(See the Pathophysiology of Platelets section in Chapter 21 and related articles in the Reference Guide appendix on the CD.)

Hemorrhagic diseases, conditional violations coagulation systems blood

The stages of the hemocoagulation cascade are presented in Fig. 21-25.

Kinds coagulopathies

Hereditary coagulopathies.

Deficiency of factor VIII components (hemophilia A, disease background von Willebrand ) and factor IX (hemophilia B); these are the most common hereditary coagulopathies (more than 95% of cases). See the article “Hemophilia” in the Terms Reference appendix on the CD-ROM.

Deficiency of factors VII, X, V and XI (0.3–1.5% of cases each).

Deficiency of other factors: XII (defect Hageman), II (hypoprothrombinemia), I (hypodysfibrinogenemia), XIII (fibrin-stabilizing factor deficiency) are extremely rare (single observations).

Acquired coagulopathies.

DIC syndrome.

Deficiency or inhibition of the activity of prothrombin complex factors (II, VII, X, V) in liver diseases, obstructive jaundice, intestinal dysbiosis, overdose of vitamin K antagonists (coumarins, phenylin), hemorrhagic disease of newborns.

Coagulopathies associated with the appearance in the blood of immune inhibitors of coagulation factors (most often antibodies to factor VIII).

Bleeding caused by heparinization, administration of fibrinolytic drugs [(streptokinase, urokinase, alteplase (actilyse)] and defibrinating action.

Hemorrhagic syndromes are a group pathological conditions, characterized by increased

bleeding.

Bleeding occurs as a result of pathology:

1. Platelets;

2. Blood clotting;

3. Walls of blood vessels.

Blood coagulation is a complex of sequential reactions involving platelets, plasma and tissue factors and leading to the formation of a fibrin clot at the site of vessel damage. Damage to a vessel entails a number of processes: 1 - contraction of the damaged vessel; 2 - accumulation of platelets at the site of injury; 3 - activation of coagulation factors; 4 - activation of the fibrinolysis reaction.

Blood coagulation itself consists of three main stages: 1) the formation of an enzyme that activates prothrombin; 2) conversion of prothrombin to thrombin under the influence of an enzyme that activates prothrombin; 3) conversion of fibrinogen to fibrin under the influence of thrombin. Blood coagulation can occur both inside the vessel and therefore it is called internal coagulation, and outside the vessel. Coagulation outside the vessel is called extrinsic coagulation, and active clotting factors are released primarily from damaged tissue.

Internal coagulation occurs without the influence of tissue damage factors and, strange as it may seem, blood coagulating on the surface of a glass or inside a test tube coagulates according to a mechanism identical to internal coagulation. Internal coagulation occurs when blood comes into contact with negatively charged surfaces. In conditions of an intact vessel, coagulation occurs when blood comes into contact with negatively charged endothelial surfaces, as well as with collagen or phospholipids. Negative charges, collagen or phospholipids activate factor XII (Hageman factor), which in turn activates factor XI. Then activated factor XI in the presence of Ca2+ ions and phospholipids makes factor IX (Christmas factor) active. Factor IX forms a complex with factor VIII (antihemophilic factor), Ca2+ ions and activates factor X (Stewart factor). Activated factor X interacts with activated factor V, Ca2+ ions and phospholipids, resulting in the formation of an enzyme that activates prothrombin. The reaction then proceeds similarly for both internal and external coagulation.

External coagulation occurs under the influence of factors released from damaged tissues. The initial moment of external coagulation is the interaction of factor III (tissue factor) in the presence of Ca2+ ions and phospholipids with factor VIII (proconvertin), which leads to the activation of factor X (Stewart factor). Further, the reaction proceeds similarly to the reaction that occurs during internal coagulation.

The second stage of coagulation proceeds similarly to both internal and external coagulation and consists in the fact that factor II (prothrombin) interacts with factors V and X and leads to the formation of thrombin.

The third stage of coagulation involves the conversion of fibrinogen to fibrin under the influence of thrombin. Thrombin breaks down fibrin; the resulting fibrin monomer in the presence of Ca2+ ions and factor XIII (fibrin-stabilizing factor) forms dense filaments in the form of an insoluble clot. The resulting clot (thrombus) contracts (compacts), releasing a liquid - serum. In the process of compaction, called retraction, platelets play an important role. In conditions of platelet deficiency, the retraction process does not occur. The fact is that platelets contain a contractile protein - thrombostenin, which contracts when interacting with ATP. The blood clot is not permanent and after some time it dissolves or lyses. During the process of lysis, the inactive precursor profibrinolysin (plasminogen) is converted into active form- fibrinolysin (plasmin) under the influence of factor XII (Hageman factor). The first portions of fibrinolysin that appeared according to the principle feedback enhance the influence of factor XII on the process of formation of fissionable

enzyme. Fibrinolysin destroys the network of fibrin threads and leads to the disintegration of the clot. The body maintains a balance between the process of blood clot formation and their lysis. So, in response to the introduction natural factor, which prevents clotting - heparin, the concentration of profibrinolysin increases in the blood. Most likely, this process is responsible for maintaining normal blood flow in the capillaries, preventing the formation of microthrombi.

Our body contains factors that control, or rather inhibit, the process of blood clot formation. The blood contains factors such as antithromboplastin, antithrombin and , which can control coagulation at various stages. So, if a small amount of thromboplastin is released from the tissues, then it may be enough to start the coagulation process and activate the X factor. However, in this case, coagulation can be prevented by the influence of heparin.

A slowdown in the coagulation process can be observed: 1) with a decrease in vitamin K in food, which reduces the level of prothrombin; 2) with a decrease in the number of platelets, which reduces the level of coagulation-activating factors; 3) for liver diseases leading to a decrease in the content of proteins involved in coagulation and coagulation activation factors; 4) for diseases of a genetic nature, when the body lacks some coagulation factors (for example, VIII - antihemophilic); 5) with an increase in fibrinolysin content in the blood.

An increase in coagulability can be observed: 1) with local suppression of the activity of antithrombin and thromboplastin (C-activated protein) in small vessels, most often this process occurs in small venous vessels and leads to the formation of blood clots; 2) for major surgical interventions that cause extensive tissue damage; 3) with severe vascular sclerosis, due to an increase in charges in the vascular wall (endothelium) and the accumulation of Ca2+ ions on it.

Individual episodes of increased bleeding can be separated from each other by many months or even years, and then occur quite often. In this regard, for the diagnosis of hereditary hemostasis defects, a carefully collected anamnesis is extremely important not only for the patient himself, but also for all his relatives. In this case, it is necessary to find out the type of bleeding in the patient and relatives by carefully analyzing the minimal signs of bleeding - periodic: skin hemorrhagic syndrome, nosebleeds or bleeding after injuries, cuts, duration of menstrual bleeding in girls, hemarthrosis, etc.

IN clinical practice It is advisable to distinguish several types of bleeding:

1. Hematoma type - painful extensive hemorrhages are determined in the subcutaneous tissue, under the aponeuroses, in the serous membranes, in the muscles and joints, usually after injuries with the development of deforming arthrosis, contractures, and pathological fractures. Prolonged, profuse post-traumatic and postoperative bleeding is observed, and spontaneous bleeding is less common. The bleeding is late in nature, several hours after the injury. The hematoma type is characteristic of hemophilia A and B (deficiency of factors VIII and IX).

2. Petechial-spotted (bruised), or microcirculatory, type is characterized by petechiae, ecchymoses on the skin and mucous membranes, spontaneous (occurring mainly at night asymmetric hemorrhages in the skin and mucous membranes) or bleeding that occurs at the slightest injury: nasal, gingival, uterine, renal. Hematomas are rarely formed, the musculoskeletal system is not affected. Postoperative bleeding is not observed (except for tonsillectomy). Cerebral hemorrhages are frequent and dangerous; as a rule, they are preceded by petechial hemorrhages into the skin and mucous membranes.

The microcirculatory type is observed with thrombocytopenia and thrombocytopathies, with hypo- and dysfibrinogenemia, and deficiency of factors X, V and II.

3. Mixed (microcirculatory-hematoma) type is characterized by a combination of the two above forms and some features; The microcirculatory type predominates, the hematoma type is slightly expressed (hemorrhages mainly in the subcutaneous tissue). Hemorrhages into the joints are rare. This type of bleeding is observed in von Willebrand disease and von Willebrand-Jurgens syndrome, since a deficiency of the coagulant activity of plasma factors (VIII, IX, VIII + V, VII, XIII) is combined with platelet dysfunction. Of the acquired forms, this type of bleeding can be caused by a deficiency of the factors of the prothrombin complex and factor XIII, disseminated intravascular coagulation syndrome, an overdose of anticoagulants and thrombolytics, and the appearance of immune inhibitors of factors XIII and IV in the blood.

4. Vasculitic purpuric type is caused by exudative-inflammatory phenomena in microvessels against the background of immunoallergic and infectious-toxic disorders. The most common disease in this group is Henoch-Schönlein syndrome. Hemorrhagic syndrome is represented by symmetrically located elements, mainly on the extremities in the area of ​​large joints, clearly demarcated from healthy skin, protruding above its surface, represented by papules, blisters, vesicles, which may be accompanied by necrosis and the formation of crusts. There may be a wave-like flow, a “blooming” of elements from crimson to yellow color followed by slight peeling of the skin. With the vasculitic purpuric type, abdominal crises with heavy bleeding, vomiting, macro- and microhematuria (more often) are possible, often transforming into DIC syndrome.

5. The angiomatous type is characteristic of various forms of telangiectasia with angiomas, arteriovenous shunts. The most common type is Osler-Rendu syndrome.

Disorders in the megakaryocyte-platelet system:

1. Change in the quantitative composition of platelets - thrombocytopenia;

2. Changes in the functional properties of platelets - thrombocytopathy.

Thrombocytopenia:

The most well-known and common disease in this group is idiopathic (ITP). Idiopathic can be either hereditary or acquired, the latter option being more common. The incidence of ITP is approximately 1/10,000, and women are affected approximately 2 times more often than men (in childhood, girls and boys are affected with the same frequency). Acquired ITP most often affects women aged 20 to 50 years. ITP often occurs against the background of diseases such as chronic lymphocytic leukemia, lymphogranulomatosis, systemic lupus erythematosus and others autoimmune diseases. In this case main reason a decrease in the number of platelets in the blood is an inhibition of the megakaryocyte lineage bone marrow, however, the addition of an immune mechanism increases thrombocytopenia.

in ITP, they are produced against unchanged platelet proteins; most often they are directed against the main and most immunogenic platelet proteins - the complex of membrane glycoproteins (GP) IIb-IIIa and GP Ib. The pathogenesis of the disease is based on a shortening of the lifespan of platelets. The normal lifespan of platelets reaches 7-10 days, and with Werlhof's disease (ITP) it is several hours. As a result of the destruction of platelets (destruction occurs both due to the action of autoantibodies and due to destruction in the spleen), a sharp activation of thrombocytopoiesis occurs. The formation of platelets increases 2-6 times, the number of megakaryocytes increases, and the detachment of platelets from megakaryocytes sharply accelerates. All this creates the erroneous impression that platelet lacing is impaired, although in fact the lacing occurs much faster.

The so-called hapten (heteroimmune) thrombocytopenias are classified into a separate subgroup. In this pathology, autoantibodies are produced against altered or foreign antigenic structures on the surface of platelets, appearing, for example, in as a result of exposure to drugs or viruses. Among, capable of stimulating the production of haptens and drug-induced thrombocytopenia, secrete drugs of the quinidine series and. Drug-induced thrombocytopenias are temporary and usually disappear after stopping the medication. Heteroimmune thrombocytopenia as a result of exposure to viruses most often occurs in children; sometimes thrombocytopenia can be a consequence of vaccination. In adults, there is a known association of thrombocytopenia with HIV infection. It is assumed that viruses can change antigenic structure platelets, both as a result of interaction with membrane proteins and due to nonspecific fixation on the surface of platelets. As a result, production against altered platelet and cross interaction actual antiviral antibodies with platelets.

Thrombocytopathies:

Thrombocytopathies are a large group of diseases characterized by a qualitative disorder blood platelets(platelets). In thrombocytopathies, platelet aggregation and adhesion functions are reduced or absent altogether, therefore the main symptom of thrombocytopathies is increased bleeding. Thrombocytopathies occur with equal frequency in both men and women, but signs of increased bleeding in women are more pronounced.

Thrombocytopathies are divided into two large groups: congenital and acquired during life.

Congenital platelet disorders are characterized by a hereditary deficiency of special proteins on the surface of platelets or protein granules located in the cavity of the platelets, as well as a violation of the shape and size of platelets. Congenital thrombocytopathies include: Glanzmann thrombocytopathy, Bernard-Soulier syndrome, May-Hegglin anomaly, as well as extremely rare platelet defects such as Pearson-Stobe anomaly, essential atrombia, etc. The most dangerous manifestations of the disease are hemorrhages in the retina, brain and its shells.

With acquired thrombocytopathies, as with congenital ones, there is a deficiency of proteins, both on the surface of platelets and in the platelets themselves, but the cause of this is external influence. Clinical manifestations are the same as for congenital thrombocytopathies.

Causes of acquired thrombocytopathies:

diseases of the blood system (acute leukemia, chronic leukemia, various forms of coagulation factor deficiencies);

liver diseases, most often the liver;

kidney diseases with impairment of their functions in late stages (severe forms glomerulonephritis, polycystic disease, etc.);

· massive blood and plasma transfusions (in this situation, changes in platelets are eliminated, as a rule, on their own within 2-3 days);

· diseases of the thyroid gland, such as, the treatment of which completely restores platelet function;

· use of various medications: derivatives of non-steroidal anti-inflammatory drugs, such as ortofen; as well as trental, , , some psychotropic drugs - , etc. The time of action on platelets of each of the drugs depends on the time of circulation in the blood, the effect of some of them, for example, aspirin, can last up to 5 days after a single dose. The severity of increased bleeding is strictly individual for each person.

The treatment uses drugs that enhance the adhesive ability of platelets (these are PAMBA, ATP, hormone therapy). Used for massive bleeding replacement therapy blood products: fresh frozen plasma, cryoprecipitate, in rare cases, donor platelet concentrate. In acquired thrombocytopathies, the main task is to eliminate the cause of increased bleeding.

Coagulopathies:

1. Hemophilia.

2. Pseudogemophilia (hypoprothrombinemia, hypoproaccelerinemia, hypoconvertinemia, afibrinogenemia, etc.)

3. Hemorrhagic diseases caused by excess blood anticoagulants.

Hemophilia.

Hemophilia A is caused by a deficiency of antihemophilic globulin (coagulation factor VIII). Hemophilia B is caused by a deficiency of coagulation factor IX. Hemophilia A and B are hereditary diseases, transmitted linked to the X chromosome, autosomal recessively). Hemophilia C is caused by a deficiency of coagulation factor XI (Rosenthal factor) and is the most severe. Hemophilia C is transmitted in an autosomal dominant manner (both women and men are affected).

The basis for increased bleeding is a deficiency of blood clotting factors. The provoking moment is always trauma (physical), i.e. violation of the integrity of the mucous membrane and skin.

The course of hemophilia has the following features: 1) bleeding does not occur immediately after injury, but 2-3 hours later; 2) the severity of bleeding is inadequate to the injury; 3) duration of bleeding up to several hours and days; 4) a clot forms, but the blood continues to flow (leaks); 5) there is bleeding in the muscles, joints, internal organs; 6) bleeding can be from the same place (bleeding recurs).

Treatment: for hemophilia A: transfusion of citrated blood, cryoprecipitate; for hemophilia B, C, transfusions of blood plasma, cryoprecipitate, and coagulation factor concentrate are used. For hemarthrosis, the joint is punctured with injection to prevent hemarthritis from developing.

Rare coagulopathies.

The remaining 15% represent less common deficiencies of other factors of the hemostatic system: fibrinogen, prothrombin, factors 5,7,10,11,13, and combined deficiency of factors 5 and 8. Clinical manifestations of these “rare disorders” include the entire range from the trend to bleeding to serious and even life-threatening bleeding.

Fibrinogen is a glycoprotein with a molecular weight of 340 kDa, which is synthesized in the liver and plays a key role in the hemostatic system. Its complete absence, called congenital afibrinogenemia, leads to both minor and very severe bleeding. The disease is transmitted according to an autosomal recessive pattern with a frequency of 1-2 cases per million people and is expressed in a decrease in fibrinogen levels to 200-800 mg/l. Symptoms usually appear from the first days of life in the form of bleeding in umbilical region. As you get older, bleeding can occur anywhere and have catastrophic consequences. Congenital hypofibrinogenemia is manifested by mild or moderate bleeding, which can, however, only be stopped with serious therapeutic measures; acquired - can be observed with the syndrome, liver diseases and against the background of the use of thrombolytics.

Congenital prothrombin deficiency is an extremely rare autosomal recessive disorder. All patients had both external bleeding from the mucous membranes and internal various localizations, intracranial hematomas are common. Hereditary prothrombin deficiency is also described as a mixed deficiency of factors 7, 9, 10 and proteins C and S. There are also cases of congenital dysprothrombinemia, in which the severity of the tendency to bleeding corresponds to the level of prothrombin in the blood.

Factor VII is converted to its active form by tissue factor (TF), and the TF:VIIa complex initiates the coagulation process. F VII deficiency is rare pathology(1 case in 500,000 people), and its symptoms vary. Patients with factor activity less than 1% suffer from severe coagulation disorders similar to severe hemophilia A. If Factor VII levels are greater than 5%,

Patients experience only minor episodes of bleeding of the mucous membranes. There are reports of the possibility of thrombosis due to F VII deficiency, and a combined deficiency of factors VII, IX and X has also been described.

Factor X is a 2-chain zymogen with a molecular weight of 56 kDa that circulates freely in plasma. Its activated form is an enzymatic component of the prothrombinase complex, which is a catalyst in the process of conversion of prothrombin to thrombin. Congenital deficiency of this factor is rare and is transmitted through an autosomal recessive mechanism. Acquired deficiency has been described in amyloidosis, acute respiratory infections and leukemias such as acute myeloblastosis. The type and frequency of bleeding usually corresponds to the level of factor deficiency. The most frequent symptoms include hemarthrosis, menstrual bleeding and spontaneous hematomas, hematuria and lesions of the central nervous system are less common.

Factor XI deficiency presents with extremely varied clinical presentations, but bleeding usually occurs only in association with surgery or trauma. In women, this pathology occasionally manifests itself as profuse menstrual bleeding. The deficiency is inherited by an autosomal recessive mechanism and is most often found among Ashkenazi Jews, where the incidence of the pathology reaches 8%. Unlike classical hemophilia, with this disease there is no correspondence between the level of factor in the blood and the severity of bleeding.

Vasopathies.

The most common is Henoch-Schönlein disease (hemorrhagic immune microthrombovasculitis). The pathogenesis of Henoch-Schönlein disease is based on the immune system. pathological process. In fact, with this pathology we're talking about about the 3rd type of allergic reactions - the Arthus phenomenon. This disease is considered hereditary and is inherited in an autosomal recessive manner. Occurs with equal frequency in boys and girls. More often, episodes of this disease are observed in spring and autumn. More often in children of preschool and school age. The process of vascular damage can be systemic in nature, but most often (in the vast majority of cases), it is localized within one particular system.

Clinical manifestations:

· cutaneous form characterized by the appearance predominantly on the extensor and lateral surfaces of the lower extremities, buttocks and rarely on the torso of a hemorrhagic rash, the size of which ranges from a millet grain to a 5-kopeck coin. Macular-papular rash. Sometimes the rash is itchy. The elements are combined with swelling of the lower leg, feet, external genitalia, eyelids, similar to Quincke's edema. Hemorrhagic elements in severe cases become necrotic, which is one of the criteria for the severity of the disease. After some time, the elements disappear, and then a relapse occurs. A relapse can be triggered by a violation of the motor pattern, diet, etc.

· articular form: large joints are most often involved in the pathological process. The basis of the articular syndrome is the leakage of serous-hemorrhagic fluid into the cavity and synovium, periarticular tissue. Clinic of articular syndrome: large joints are more often affected, externally the joints are enlarged, pain syndrome and all this passes without any consequences. At the height of the process, pain is very disturbing, the patient takes a forced position. The joints are swollen and painful on palpation.

abdominal form: it is based on the leakage of serous-hemorrhagic fluid into the mucous membranes digestive tract. Clinic: abdominal pain, development of protective tension in the anterior abdominal wall. Dyspeptic symptoms: vomiting, sometimes with blood, loose stools with blood. Such children most often end up with surgeons or in an infectious diseases hospital with a clinical picture of some kind of gastroenteritis. If the child is admitted to the surgical department, the diagnosis is made during laparotomy.

· unfortunately, neurogenic symptoms are also possible with this disease, when there is hemorrhage in the structures of the brain or spinal cord. In this case, it is possible that peripheral paralysis, aphasia, epileptiform seizures.

· also describe the cardiac form, when there are characteristic changes in the membranes of the heart. In this case, an expansion of all boundaries of the heart and a decrease in blood pressure are observed.

Clinical blood test: decrease in the amount of hemoglobin, color index, anisocytosis, poikilocytosis, leukocytosis, neutrophilia with a shift to the left, accelerated ESR. Protein fractions: decrease in alpha globulins, increase in some fractions. Study of blood clotting time - reduced.

In all cases, regardless of the severity of the disease, heparin is prescribed at a dose of 150-500 IU/kg/day in four doses. Heparin is injected subcutaneously in the abdominal area. At mild form heparin is administered for 10-14 days. At medium degree severity and in severe cases, heparin is administered intravenously for the first 2-3 days, and then subcutaneously for 1-1.5 months. The dose is selected individually; the criterion for effectiveness is the clotting time, which should decrease. For moderate and severe forms, 0.5 - 0.7 mg per kg per day is also used for 5 days, then a 5-day break is taken, then another course of 5 days is carried out, and so on until the effect is achieved. In some cases, especially in chronic recurrent forms, drugs of the aminoquinoline series can be used: resokhin, plaquinil, etc. they have an immunosuppressive, anti-inflammatory, analgesic effect. Also, for moderate and severe forms, antiplatelet agents (trental) can be used. You can also use plasmapheresis, enterosorption.

Rendu-Osler disease.

Associated with pathology of the vascular wall, resulting in the formation of hemangiomas. It is based on a defect in the wall, a change in its subendothelial layer. The wall not only does not participate in hemostasis, but is also easily injured. As a rule, the disease is hereditary. Inherited recessively. Most often it appears on the oral mucosa, gastrointestinal tract, facial skin, lips. It goes through three stages in its development - the speck, star and hemangiomas stage. A diagnostic feature is that these formations turn pale when pressed. The clinical picture is characterized by angiomatous type of bleeding. The state of the platelet link is normal, plasma factors are normal. Therapy is symptomatic - in some cases, these formations can be excised.

Hemorrhagic syndrome is a violation of the hemostatic system - one of the main biological processes in the body. The main functions of this system are to preserve blood in a liquid state and stop internal bleeding when the vascular walls are damaged. When the integrity of the vessel is compromised, blood penetrates beyond its boundaries, and a specific red and bluish coloration appears on the skin. In the photo below, the patient hemorrhagic syndrome looks like part of his body is covered in bruises and red marks. Of particular danger are hemorrhages of the mucous membranes and internal bleeding.

The disease develops at any age. According to statistics, women are more susceptible to it than men. In the pathological course of pregnancy it occurs in newborns.

Treatment of the syndrome involves A complex approach, which is based on therapeutic methods aimed at improving the blood clotting process. In some cases, when there is a risk of loss large quantity blood, it is important to provide immediate medical attention.

Reasons for the development of pathology

Hemorrhagic syndrome occurs due to disruption of complex hematopoietic processes.

In the presence of certain diseases, there is a high probability of formation of this syndrome. At risk are patients diagnosed with:

• hepatitis - inflammatory processes in the liver;
• cirrhosis of the liver, when the organ is not able to produce substances involved in the process of blood clotting;
• oncological neoplasms;
• infectious and viral diseases;
• hemorrhoidal syndrome;
• hemophilia - a condition of the body associated with a violation of the coagulation process (blood clotting);
• vasculitis – inflammation of blood vessels of an immunopathological nature;
• thrombocytopathy – with a sufficient platelet content, a violation of their functions is observed;
• thrombocytopenia – while platelet functions are preserved, their number decreases.

In medical practice, there have been cases where changes in the process of blood clotting occur under the influence of certain medications.

Often, hemorrhagic syndrome develops in newborns. It usually appears a few days after the baby is born. Among the factors influencing its formation are:

• long-term use expectant mother certain medications, the components of which are able to penetrate to the fetus through the placenta (anticonvulsants, antituberculosis drugs, antibiotics, anticoagulants, phenobarbital);
• birth of a child before the due date;
• fetal hypoxia;
• the mother has diseases that are at risk.

The causes of hemorrhagic syndrome are also hereditary and associated with gene mutation.

Classification and main features

IN medical science There are two forms of the syndrome:

• primary (congenital), due to a person’s genetic predisposition;
• secondary (acquired), arising due to damage to blood vessels of various types.

Depending on what part of the body the lesion is located and what caused it, several types of the disease are distinguished. Each of them has characteristic symptoms and features of the therapeutic technique.

Diagnostic tests

The main goal of diagnostics is to establish the cause of the disease, on the basis of which it is prescribed further treatment. If a person's syndrome manifests itself in acute form, you must first stop the bleeding and stabilize the condition.

Main diagnostic method if hemorrhagic bleeding is suspected, laboratory tests are performed: a detailed blood test and a urine test, which allows you to see the whole picture. Important has an indicator of the number of platelets in peripheral blood. Additional methods examinations include simple coagulation tests and sternal puncture.
When making a diagnosis, the duration of bleeding, as well as the nature of blood clotting, are taken into account.

Therapeutic measures

Treatment of hemorrhagic syndrome in adults depends on several factors:

• causes of the disease;
• stages of development;
• the nature of the course and severity of the disease.

Urgent Care

In the acute form of hemorrhagic syndrome, emergency care may be needed. It is important to stop the source of damage. For this purpose, the following therapeutic methods are used:

• conservative hemostatic therapy;
• cryotherapy;
• laser barotherapy;
• surgical intervention.

Drugs

When the first one is rendered health care and the bleeding is stopped, the patient is prescribed drugs that have a positive effect on the coagulation process. Modern pharmaceuticals have a large number medicines, improving blood clotting.

In cases of significant blood loss, it is necessary replacement treatment, involving the introduction of plasma. The use of heparin and prednisolone is possible.

If hemorrhagic syndrome occurs against the background of any diseases, the treatment has characteristic features. Therapeutic measures are aimed not only at stopping the pathological focus, but also at eliminating the concomitant illness.
For some diseases, it cannot be guaranteed that treatment will be highly effective. For example, with a congenital type of hemophilia, it is necessary to take hormonal drugs, the action of which is aimed at normalizing the blood circulation process. Patients must be under close medical supervision and follow all his recommendations.

Home Recipes

Traditional medicine cannot replace drug therapy. However, their use has a positive effect on the condition of the walls of blood vessels. Only an integrated approach that combines all types of therapy can have the desired effect. Useful for hemorrhagic syndrome are green varieties of apples, cherries, red grapes, raspberries, strawberries, currants, sea buckthorn, red pepper, rowan, rose hips, buckwheat, yarrow.

They have a whole complex of vitamins and nutrients, strengthening blood vessels and increasing blood clotting.

Despite all the effectiveness of medicines, as well as mixtures and decoctions prepared according to folk recipes, it is necessary to exclude those components that are allergens. Please read the instructions carefully for any contraindications and side effects. If the body is individually intolerant to the components of the drug, its use is excluded.

At normal functioning hemostatic mechanisms, hemorrhages do not develop spontaneously, and injuries to most vessels (with the exception of injuries large arteries, main veins and the vascular network of parenchymal organs) do not threaten the patient’s life due to the local formation of a fibrin clot at the site of damage to the vascular bed. Numerous coagulation defects interfere with physiological performance hemostatic reactions, so when gross violations of the hemostasis system, even minor vascular damage can cause the patient’s death. It should also be taken into account that in a considerable number of patients, hemorrhagic syndrome is caused not by vascular injury, but by other causes (inflammatory process, separation of the placenta, destruction of the mucous membrane or hormonally induced rejection of the endothelium during menstruation, etc.).

Nature provided to the human body quite a wide range of concentrations for each of the coagulation factors. In addition, the level of isolated reduction in any coagulation factor that causes hemorrhage is approximately 5-10 times lower than the average normal value.

It is very important to detect a hemostatic defect as early as possible, since patients with even moderate bleeding during and/or after surgery or childbirth can lose a fairly large volume of blood. In such situations, the attending physician will no longer have the opportunity for a more precise diagnosis, since life threatening hemorrhages often induce consumptive coagulopathy, which will result in depression of most components of the hemostatic system (platelets, fibrinogen and many others), which will not allow identifying the hemostatic defect underlying uncontrolled bleeding.

In 1975 prof. Z.S. Barkagan developed a simple but very useful classification of hemorrhage variants. The author of this classification identified five main types of bleeding: hematomal, microcirculatory, mixed, vasculitic purpuric and angiomatous. In table 2.3 presents the characteristics of these five types of bleeding, and also shows in which hemorrhagic diseases one or another variant develops. Despite its simplicity, this classification reflects not only the clinical and morphological features of hemorrhages, but also pathogenetic mechanisms, which determine them, so it has been an effective diagnostic tool for about 40 years.

Causes of hemorrhagic syndrome

HS due to blood clotting disorders (coagulopathy):

• Hemophilia.
• Lack of prothrombin.
• Overdose of anticoagulants.

HS due to impaired platelet formation:

• Werlhof's disease.
• Symptomatic thrombocytopenia.
• Thrombocytopathies.

3. HS due to vascular damage (vasopathy):

• Hemorrhagic vasculitis.
• Hemorrhagic telangiectasia.

There are 5 types of bleeding

1. Hematoma type. It is characterized by massive painful hemorrhages in the muscles, as well as in large joints. This type is characteristic of hemophilia.
2. Petechial-spotted type (bruise). It is characterized by painful superficial bleeding into the skin and bruising. Hemorrhages occur with negligible trauma (for example, when measuring blood pressure). This type is characteristic of thrombocytopathy, and may be due to a lack of fibrin and certain coagulation factors (X, V, II).
3. Mixed bruise-hematoma type. It is characterized by a combination of petechial-spotted bleeding with large hematomas in the absence of hemorrhages in the joints (in contrast to the hematoma type). This type is observed with factor XIII deficiency, overdose of anticoagulants, and constitutional thrombocytopathies.
4. Vasculitic purpuric type. It is characterized by skin hemorrhages in the form of purpura. This type is characteristic of hemorrhagic vasculitis and thrombocytopathy.
5. Angiomatous type. It is characterized by repeated bleeding of a certain localization. This type is observed with telangiectasias and angiomas.

Petechia is a small spot (1-3 mm) of a certain shape of purple-red color. Does not disappear when pressed.
Purpura is a subcutaneous layer of tissue, altered due to hemorrhage, purple or red-brown in color, easily visible through the epidermis.
Echimosis is a hemorrhagic spot (larger than petechiae) of blue or purple color.

Symptoms and signs of hemorrhagic syndrome

The clinical picture consists of symptoms of bleeding of various locations and hemorrhagic skin rashes.

Bleeding may occur spontaneously or under the influence external factors: hypothermia, physical stress, minor injuries. Skin manifestations hemorrhagic diathesis is diverse - from small petechial rashes and bruises to hemorrhages merging with each other with an ulcerative-necrotic surface. Manifold clinical manifestations syndrome corresponds to five types of bleeding.

Hemorrhagic manifestations can be combined with articular syndrome (arthralgia, hemarthrosis), abdominal syndrome, there may be an increase in temperature.

Hemorrhagic syndrome can be observed in diseases such as malignant neoplasms, hepatitis and cirrhosis of the liver, leukemia, sepsis, systemic diseases connective tissue, severe infections, etc. In such cases clinical picture will consist of the symptoms of the underlying disease and the symptoms of hemorrhagic diathesis.

Laboratory and instrumental studies

1. Expanded clinical analysis blood with platelet count.
2. Blood clotting time.
3. Duration of bleeding.
4. Blood clot retraction time.
5. Blood for prothrombin and fibrinogen.
6. Plasma tolerance to heparin.
7. General urine analysis.
8. Sternal puncture according to indications.

If necessary, the scope of laboratory tests can be expanded (thrombin and prothrombin time, determination of the activity of coagulation factors, determination of antihemophilic globulin, study of the adhesive-aggregation function of platelets, recalcification time).

Stages of diagnostic search

1. basis diagnostic algorithm is the presence of hemorrhagic syndrome. For this purpose, when collecting complaints, it is necessary to clarify the location and nature of bleeding, the frequency of bleeding, find out whether the bleeding is associated with any provoking factor or occurs spontaneously, etc.
2. The second stage is the history and physical examination. If the disease manifests itself with early childhood, it is natural to assume that the disease is hereditary or congenital (in this case, it is important to collect information about the presence of similar symptoms in close relatives).

Since most acquired hemorrhagic diathesis are symptomatic, it is necessary to clarify the presence of the underlying pathology. These may be liver diseases, leukemia, radiation sickness, aplastic anemia, systemic connective tissue diseases. Bleeding may be associated with the action of drugs that affect platelet function (aspirin) or blood clotting (anticoagulants).

An objective examination involves a thorough examination skin, which will allow us to determine the nature and type of hemorrhagic manifestations. Inspection of the mucous membranes is mandatory, since hemorrhages can be located not only in the skin, but also in the mucous membranes. In some cases, angiectasias can be detected in the form of vascular spiders or bright red nodules protruding above the surface of the skin. They are characteristic of hereditary vascular dysplasias (Rendu-Osler disease) or can be acquired (for example, with cirrhosis of the liver).

Enlargement of joints with limited mobility occurs in hemophilia. These disorders are a consequence of bleeding in the joints (hemarthrosis).

With hemorrhagic vasculitis there may be a slight increase lymph nodes. Autoimmune thrombocytopenia is accompanied by an enlarged spleen.

Tests for capillary resistance (fragility) may have diagnostic value:

1. Rumple-Leede-Konchalovsky symptom.
2. Pinch symptom - if you pinch the skin under the collarbone, pinpoint hemorrhages will appear. When a suction cup is applied to the skin, pinpoint hemorrhages appear. These tests are positive in thrombocytopenia, hemorrhagic vasculitis, taking anticoagulants.
3. Additional research methods will help establish a final diagnosis.

Differential diagnosis of hemorrhagic syndrome

Bleeding can be detected in diseases whose pathogenesis does not involve a defect in the hemostatic system, so the greatest difficulty in examining such patients is to determine the etiology of the hemorrhagic syndrome. In case of defects in the hemostatic system, hemorrhages of different localization and other clinical manifestations of hemostatic dysfunction are usually combined with each other (for example, epistaxis and uterine bleeding), forming an idea of ​​the systemic nature of manifestations, which is very characteristic of most hemorrhagic diseases. In addition, with hemorrhagic diseases and syndromes, recurrent hemorrhages are often combined with chronic posthemorrhagic anemia. However, in a number clinical cases There are also local hemorrhagic manifestations without other symptoms of bleeding and iron deficiency. In the presence of local hemorrhages or asymptomatic bleeding, it is only possible to exclude a hemorrhagic disease laboratory test hemostasis systems. If one or two clinical manifestations of bleeding are detected, it is useful to consider their relationship with a hemorrhagic disease.

When conducting differential diagnosis various types hemorrhagic diathesis should be guided by the following considerations.

1. Identification of coagulopathies from the group of hemorrhagic diathesis is based on clinical features bleeding (hematoma type, often hemarthrosis, delayed bleeding), laboratory coagulation studies (extension of clotting time, thrombin, prothrombin and thromboplastin time), as well as negative results tests for capillary fragility.
2. Hemorrhagic diathesis due to impaired platelet formation is characterized by a petechial-spotted type of bleeding and bleeding from the mucous membranes, positive tests for capillary fragility (symptom of a tourniquet, pinch), a decrease in the number of platelets or their functional failure.
3. Vasopathies are diagnosed by vasculitic purpuric or angiomatous type of bleeding and unchanged laboratory data.
4. The diagnosis of hereditary forms is based on the study of family history and laboratory data.
5. The diagnosis of symptomatic forms is based on identifying the clinical signs of a particular (main) disease, taking into account the characteristics of the manifestation of hemorrhagic syndrome. Other purchased hemorrhagic diathesis can be diagnosed only after excluding symptomatic variants.

Paramedic tactics for hemorrhagic syndrome

1. In case of bleeding, provide emergency assistance to the extent necessary and possible.
2. If a patient with hemorrhagic syndrome is identified, give a referral for consultation with a doctor.
3. After establishing a diagnosis, the paramedic monitors patients subject to medical examination, working in contact with the doctor.
4. The paramedic must know all the patients in his area who suffer from hemophilia and have an understanding of modern ways its treatment (coagulation factor VIII concentrate - in mild cases 10-15 units/kg, in more severe cases 25-40 units/kg per course of treatment; for hemorrhages in the cavity of the skull, chest, abdomen, the initial dose is 40-50 units. /kg).