Система Orphus

Symptoms of disease - violation of the blood coagulability

The blood coagulation system (synonym system hemocoagulation) - multi-enzyme system that, when enabled, dissolved in the blood plasma fibrinogen is after the splitting of boundary value peptides polymerization and forms in the blood vessels fibrin blood clots, stop the bleeding.

In physiological conditions in the blood clotting system, blood balanced processes activation and inhibition, as a result of stored liquid state of the blood. Local activation of blood coagulation system, which takes place in places of damage to blood vessels, helps to stop bleeding. Activation of blood coagulation system in combination with the aggregation of blood cells (platelets, erythrocytes) plays a significant role in the development of local thrombosis during the disturbances of hemodynamics and rheological properties of blood and changes of its viscosity, inflammatory (for example, when vasculitis) and dystrophic changes of the walls of blood vessels. Multiple recurrent thrombosis in persons of young and middle age may be associated with congenital (inherited) anomalies of blood coagulation and fibrinolysis system, primarily to a decline in activity of the main physiological anticoagulants (antitrombina III and protein C and S, etc.), necessary to maintain the circulating blood in the liquid state.

Important physiological function of the blood coagulation system consists also in the fact that it by a massive thrombosis of microvessels in the zone of inflammation, around the fires of infectious diseases destruction of tissue, as well as aseptic necrosis demarcates these sites, prevents the dissemination of the infection weakens the inclusion in the overall flow of bacterial toxins and products of the decay of tissues. Together with the excessive and too widespread disseminated clotting of blood leads to the development of thrombohemorrhagic syndrome - the pathological process, which is an important component in the pathogenesis of a large number of diseases, critical and terminal conditions. In connection with the recognition of violations of blood clotting disorders and their treatment are of great importance in the medical practice.

The process of blood clotting is implemented multi-stage interaction of the phospholipid membranes («matrix») plasma proteins called clotting factors blood (blood clotting factors indicate Roman numerals; if they go into the activated form, to the number of factors add the letter «a»). The composition of these factors include proenzymes, becoming after the activation of the proteolytic enzymes, proteins, non-enzymatic properties, but necessary for fixation on the membranes of and interaction between the enzyme factors (factor VIII and V); main substrate of the blood coagulation system - fibrinogen (factor I), protein inhibitors of blood clotting, or primary physiological anticoagulants; non-protein components (the most important of them are the ions of calcium). The blood coagulation system at different levels closely interacts with the cell hemostasis, which involves endothelium of blood vessels, platelets, red blood cells, macrophages; plasma enzymatic systems, for example, kallikrein-kinin, fibrinolytic, complement, as well as the immune system.



Which diseases there is violation of the blood coagulability

Blood clotting disorders most often manifested in the form of bruises, arising spontaneously or with minor injuries. Especially, if they are combined with recurrent epistaxis or bleeding from other places. Also, increased bleeding gums, there are cuts, wounds.

To violations of blood clotting lead a number of diseases of the blood and blood vessels. This can manifest itself in the form of large bruises and with difficulty staying bleeding, and small multiple bruises in the form of a rash.

All of these diseases are serious enough (some are even dangerous for life, especially in the absence of treatment) and require medical attention.

Blood clotting disorders, characterized by anticoagulation, can be caused by deficiency of one or several coagulation factors the blood, the appearance in the circulating blood of their immune inhibitors, i.e. antibodies to factors such as blood clotting (more factors VIII, IX, V and von Willebrand factor), and the action of anticoagulants and thrombolytic drugs, the syndrome of disseminated intravascular coagulation (DIC-syndrome). Approximate distinction of the majority of these violations is possible on the basis of family and personal medical history: a type of bleeding; background diseases and impacts (including medicines), which may be related to the development of haemorrhages. Also use the results of the following laboratory tests - determination of the activated partial (partial) thromboplastin time, prothrombin and thrombin time, agglutination test platelets under the influence of ristomycin (important test for the detection of most forms of von Willebrand disease), research content in plasma fibrinogen and products of its metabolization (soluble complexes fibrin-monomer, which are revealed with the help of steam-coagulation tests, such as ethanol, protamine sulfate test, test gluing of staphylococci) and products of the cleavage of fibrinogen (fibrin) plasmin (fibrinolysin). Determination of soluble complexes fibrin-monomer and degradation products fibrinoguena especially important for the diagnosis of intravascular blood coagulation, including thrombohemorrhagic syndrome. Are also important identification fragility of vessels (for example, cuffed sample Konchalovsky - Tiller - Leede), bleeding time, count the number of platelets in the blood and investigation of their aggregational functions. The choice of diagnostic tests is determined by the data of anamnesis, clinical picture, type of bleeding, underlying diseases and impacts. After indicative are differentiating (correctional) tests.

Among the hereditary disorders of blood coagulability the vast majority of accounts for hemophilia A and b, as well as von Willebrand disease. They are also characterized by bleeding, which arose as a child; in this case there are gematomny type of bleeding (bleeding into joints and affection of the musculoskeletal system) among males in haemophilia and mixed type (petechial-spotted with rare haematomas) the persons of both sexes with von Willebrand disease. Tema laboratory symptom of these diseases is isolated extension of the blood clotting in a test of determination of the activated partial thromboplastin time under normal prothrombin and thrombin time. In von Willebrand disease often considerably elongated time of bleeding, there is a violation of platelet aggregation under the influence of ristomycin.

Isolated violation of the only prothrombin time with mixed type of bleeding is typical for hereditary of factor VII or for the early phase of application of indirect anticoagulants (coumarines, warfarin, etc.). At the complex deficit of all of vitamin K-zawisimah factors (VII, IX, X and II), observed with hemorrhagic disease of the newborn, diseases of the liver and receiving anticoagulants of indirect action, as well as for inherited deficiency of factor X, V, II, violated as a test of the activated partial thromboplastin both time and prothrombin index, but remains normal thrombin time.

Violation of the testimony of all coagulation tests, including thrombin time, is characteristic of thrombohemorrhagic syndrome, hereditary Hypo - and disfibrinogenemy, chronic lesions of the liver. At deficiency of factor XIII the testimony of all coagulation tests remain normal, but fibrin clot dissolves in 5-7 M urea.

Blood clotting disorders, characterized by a tendency to recurrent thrombosis of vessels and heart attacks bodies, often associated with hereditary or secondary (symptomatic) deficit antitrombina III - the main inactivator all the enzymes of blood clotting factors and heparin cofactor, proteins C and S (blocker activated factor VIII and V), deficiency of the components of fibrinolytic (deficit plasminogen and his endothelial activator, etc.) and kallikrein-kinin system (deficit plasma prekallikrein and the high kininogen), rarely with a deficit of factor XII and anomalies of fibrinogen. Cause thrombophilia can be and hyperaggregation platelets, the deficit of prostacyclin and other inhibitors of platelet aggregation. Secondary depletion of the above mechanisms maintaining the liquid state of the blood may be due to an intensive flow of physiological anticoagulants. The propensity to trombozam increases with the increase of blood viscosity, which is determined by the method of viscometry, as well as to increase hematocrit indicator, increased content in the blood plasma fibrinogen.

Blood clotting disorders, purpura and other hemorrhagic conditions (ICD-10), class III (2/3)
D65. Disseminated intravascular coagulation syndrome [defibrination]. Afbrinogenemia acquired. Consumption coagulopathy. Diffuse or disseminated intravascular coagulation (DJC). Fibrinolytic bleeding acquired. Purple: fibrinolytic, the lightning.
Excluded: syndrome defibrination (complicates): abortion, ectopic or molar pregnancy (O00 - O07, O08.1), the newborn (P60), pregnancy, childbirth and the postnatal period (O45.0, O46.0, O67.0, O72.3)

D66. Hereditary deficiency of factor VIII. Deficiency of factor VIII (with functional impairment) Hemophilia: RCU, And classical.
Excluded: deficiency of factor VIII with vascular disorder (D68.0)

D67. Hereditary deficiency of factor IX. Christmas disease. The deficit factor IX (with functional impairment), thromboplastic component plasma, Hemophilia In

D68. Other clotting disorders.
Excluded complicating: abortion, ectopic or molar pregnancy (O00-O07, O08.1), pregnancy, childbirth and the postnatal period (O45.0, O46.0, O67.0, O72.3)

D68.0 Von Willebrand Disease. Angiohemophilia. Deficiency of factor VIII with vascular disorder. Vascular hemophilia.
Excluded: capillary fragility hereditary (D69.8), deficiency of factor VIII: RCU (D66), with functional impairment (D66)

D68.1 Hereditary factor XI deficiency. Hemophilia With. Deficit predecessor thromboplastin plasma.

D68.2 Hereditary deficiency of other clotting factors. Congenital afbrinogenemia. Deficit: al-globulin, proaccelerin. The deficit of factor I (fibrinogen), II (prothrombin), V (labile), VII (stable), X (Stewart-Prower), XII (gene is associated), XIII (fibrin stabilizing). Dysfbrinogenemia (congenital). hypoproconvertinemia Owren's disease

D68.3 violations of Hemorrhagic caused by circulating in the blood of anticoagulants. Hyperheparinemia Increase of content of: antitrombina, anti-VIIIa, anti-Yes, anti-Xa and anti-XIa.

D68.4 Acquired deficiency of clotting factor. Deficiency of clotting factor as a result of: liver disease, deficiency of vitamin K.
Excluded: deficiency of vitamin K in the newborn (P53)

D68.8 Other refined clotting disorders. The presence of an inhibitor of systemic lupus erythematosus

D68.9 coagulation unqualified

D69. Purpura and other hemorrhagic state.
Excluded: benign hypergammaglobulinemia purpura (D89.0), krioglobulinemia purpura (D89.1), idiopathic (hemorrhagic) thrombocythemia (D47.3), the lightning purpura (D65), thrombotic thrombocytopenic purpura (M31.1)

D69.0 Allergic purpura. Purple: anaphylactoid, Henoch, netrothrombocytopenica: hemorrhagic, idiopathic, vascular. Allergic vasculitis.

D69.1 Quality defects of platelets. Syndrome Bernard-Soulier (giant platelets), Glanzmann's disease, the Syndrome of gray platelets, thrombasthenia (hemorrhagic) (inherited). Trombozitopatia.
Excluded: von Willebrand disease (D68.0)

D69.2 Other netrothrombocytopenica purpura. Purple: RCU, senile, simple.

D69.3 Idiopathic thrombocytopenic purpura. Evans Syndrome

D69.4 Other primary thrombocytopenia.
Excluded: thrombocytopenia with the lack of x-ray bone (Q87.2), transient neonatal thrombocytopenia (P61.0), Wiskott - Aldrich syndrome (D82.0)

D69.5 Secondary thrombocytopenia

D69.6 Thrombocytopenia unspecified

D69.8 Other refined hemorrhagic condition. Capillary fragility (inherited). Vascular pseudo hemophilia.

D69.9 Hemorrhagic state of the unqualified



Which doctor should I contact if there is a violation of the blood coagulability

  • Hematologist


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Do you feel disrupted blood coagulability? You should be very careful approach to your health in general. People pay not enough attention to the symptoms of the disease and don't realize that these diseases can be critically dangerous. There are many diseases that in the beginning didn't manifest in our body, but in the end it turns out, unfortunately, it have already been treated too late. Every disease has its own specific features typical symptoms - called symptoms of the disease. Definition of symptoms is the first step in the diagnosis of diseases in general. You just need a few times a year to be screened by a doctor, not only to prevent a terrible disease, but also to maintain a healthy spirit in a body and the organism in general.

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