Violation of blood clotting

Failures in the functioning of the most important body system - the system of hemostasis, designed to protect a person from loss of blood - are manifested as a violation of blood coagulability, that is, the pathology of coagulation of the protein components of the blood in case of bleeding.

The ability of the blood to clot for various reasons can decrease, leading to severe and deadly coagulopathies.

Causes of the bleeding disorders

Before highlighting the key causes of bleeding disorders, it should be recalled that coagulation in the process of stopping bleeding is a complex of complex biochemical processes caused by the action (and interaction) of almost four dozen physiologically active substances called plasma and platelet clotting factors.

In a simplified form, the process of blood clotting in damage to blood vessels can be described as the transformation of the prothrombin blood protein (plasma coagulation factor II) into a thrombin enzyme, by which the plasma in fibrinogen (a protein produced by the liver, coagulation factor I) is converted to polymerized fibrous (insoluble ) protein fibrin. The effect of the enzyme transglutaminase (XIII coagulation factor) stabilizes fibrin, and its fragments adhere to special (non-nucleated) blood elements - platelets. As a result of aggregation of platelets and their adhesion to the wall of the vessel, a blood clot forms. It is he who closes the "hole" in case of violation of the integrity of the blood vessel.

[1], [2], [3], [4], [5], [6]

Pathogenesis

The pathogenesis of abnormalities in the process of hemocoagulation is directly related to the imbalance of clotting factors, since only when activated activate the natural mechanism of formation of the blood clot and stop bleeding. No less important factor is the amount of platelets in the blood that are synthesized by the cells of the bone marrow.

The existing classification of coagulation disorders separates them - depending on the etiology - on acquired, genetically conditioned and congenital, as well as autoimmune.

Among the causes of acquired blood clotting disorders, hematologists note pathologies that lead to a decrease in the level of fibrinogen in the blood (hypofibrinogenemia) or to a decrease in the content of platelets in it:

• liver dysfunction (for example, with cirrhosis or fatty liver disease);
• thrombohemorrhagic syndrome or DIC syndrome, which develops in shock and post-traumatic states, during surgical interventions, after massive blood transfusions, in septicemia and severe infectious diseases, in the breakdown of malignant large tumors;
• deficiency of vitamin K (due to obturation of the bile duct or poor bowel function);
• pernicious (megaloblastic) anemia, arising from the lack of cyanocobalamin (vitamin B12) and folic acid (vitamin B9); this pathology can be a consequence of severe dysbiosis, as well as diphyllobothriasis (parasitism in the body of a wide ribbon);
• tumor diseases of the hematopoiesis system (leukemia, hemoblastosis) with damage to bone marrow stem cells;
• side effects of anticoagulant drugs used in the treatment of thromboses, as well as cytostatics, which are used to chemotherapy of cancer.

Read also - Acquired platelet dysfunction

The causes of bleeding disorders include the following genetic and congenital abnormalities:

• haemophilia A (deficiency of anti-hemophilic globulin-coagulability factor VIII), hemophilia B (coagulation factor deficiency IX) and hemophilia C (thromboplastin deficiency-factor XI);
• Villebrand disease (constitutional thrombopathy or Willebrand-Jurgens syndrome, when the blood lacks anti-hemophilic globulin);
• thrombocytopenic purpura (Verlhof disease);
• hereditary thrombasthenia of Glantzman;
• congenital affibrinogenemia (absence of fibrinogen in the blood) and dysfibrinogenemia (structural defects of fibrinogen molecules).

A pathogenesis of idiopathic autoimmune thrombocytopenia is associated with increased destruction of platelets in the spleen and, correspondingly, a decrease in their content in the blood.

[7], [8], [9], [10]

Symptoms of the bleeding disorders

Typical symptoms of bleeding disorders in almost all of the pathologies listed above are manifested in various combinations and with varying intensity.

The first signs are hemorrhagic syndrome - the tendency to hemorrhages in the skin and subcutaneous tissue, as well as increased bleeding of mucous membranes.

Bleeding may be capillary, hematologic or mixed. Thus, in the case of deficiency of such clotting factors as prothrombin, proaccelerin, proconvertin, Stewart-Prower factor (in von Willebrand disease, thrombocytopenia or dysfibrinogenemia), small red-violet spots appear on the skin of the legs in the region of the ankles and on the soles - petechiae (capillary hemorrhage).

If there is a deficit of antihemophilic globulin, bruises (ecchymoses) constantly appear. Subcutaneous hematomas are characteristic of hemophilia, as well as for most acquired coagulation disorders, including after prolonged use of anticoagulant drugs.

In addition, symptoms of clotting disorders are expressed in frequent spontaneous bleeding from the nose, bleeding gums, significant loss of blood during menstruation (menorrhagia), prolonged bleeding even with minor injuries to the smallest vessels. There may be frequent reddening of the eye proteins, melena (black feces) can also be observed, which indicates bleeding localized in the digestive tract. In hemophilia, blood flows not only into the tissues of the abdominal organs and muscles, but also to the joints (hemarthrosis). This causes necrosis of bone tissue to reduce the content of calcium in it and subsequent functional complications of the musculoskeletal system.

Symptoms that manifest thrombohemorrhagic syndrome, as well as its possible consequences (which can be fatal) - see Disseminated Intravascular Coagulation Syndrome (DVS).

Serious complications of most coagulation disorders are anemia, which leads to hypoxia of all body tissues, causing general weakness and decreased vitality with frequent dizziness and tachycardia.

[11], [12], [13]

Diagnostics of the bleeding disorders

Clinical diagnosis of coagulation disorders begins with the collection of anamnesis and necessarily includes laboratory studies of patients' blood.

 Such blood tests are necessary:

• general clinical;
• coagulogram (the time of blood coagulation is determined);
• PTW and PTI (prothrombin time and protitrobromine index give an idea of the speed of the coagulation process);
• TV (determines thrombin time, that is, the rate of conversion of fibrinogen to fibrin);
• ABC test (determines activated blood coagulation time);
• on the aggregation of platelets with stimulation of adenosine diphosphate (ADP);
• APTTV (determines the presence of a shortage of several plasma clotting factors);
• on antithrombin III (glycoprotein-anticoagulant blood, which inhibits the process of its coagulation).

Read more - Study of the system of hemostasis

Instrumental diagnostics (fluoroscopy, ultrasound, MRI) can be used to clarify the condition of the liver, spleen, intestine or brain.

[14], [15], [16], [17], [18]

Differential diagnosis

Given the polyetiologic nature of coagulation disorders, a real opportunity to determine the specific cause of coagulopathy and to prescribe the correct therapy is provided only by differential diagnosis.

Treatment of the bleeding disorders

With bleeding, treatment of bleeding disorders requires urgent measures - see Bleeding. Drugs that promote thrombus formation (fibrinogen, thrombin) are also used in a hospital to stop bleeding.

And the therapy of acquired coagulation disorders presupposes etiological treatment of the diseases that caused them and neutralization of provoking factors.

In many cases, the use of antihemorrhagic and haemostatic drugs is recommended: thromboplastin activating agents, heparin antagonists, fibrinolysis inhibitors, and agents that promote thrombogenesis.

Medications prescribed to stimulate the production of tissue thromboplastin (III coagulation factor) are Dicinone (another trade name is Etamsilate). This drug is used to stop capillary and internal bleeding is released as a solution for injection, and in tablets of 0.5 g for the treatment of blood clotting disorders. The dose is determined by the doctor in accordance with the blood indicators (usually 0.25-0.5 g two to three times a day), the duration of the reception also depends on the diagnosis. Dietion gives side effects in the form of headache, nausea, lowering blood pressure, skin flushing and skin sensitivity on the legs. The drug is contraindicated if increased bleeding and bruising is the result of taking anticoagulants.

Such drugs as Aminocaproic acid, Tranexam, Amben, etc., inhibit the dissolution of the blood clot (fibrinolysis). Aminocaproic acid (Epsilon-aminocaproic acid, Acycapron, Afibrine, Carpacid, Carpamol, Epsamon) in tablets of 0.5 g is given by mouth at the rate of mass, the maximum daily dose should not exceed 15 g. Side effects include dizziness, catarrhal symptoms, nausea, diarrhea. In the case of poor kidney function, aminocaproic acid is contraindicated.

Hemostatic agent Tranexam (tranexamic acid) in tablets of 0.25 g is recommended for frequent nasal bleeding and menorrhagia two tablets three times a day, but not longer than a week. Side effects and contraindications are similar to the previous drug.

When prescribing treatment for clotting disorders, a preparation containing the vitamin K analogue - Vikasol (Menadione sodium bisulfite, Menadione) is often recommended. Vikasol (in tablets of 15 mg) increases the ability of the blood to fold, acting on the production of vitamin K-dependent prothrombin and proconvertin (coagulation factors). Dosage of this drug: for adults - 15-30 mg per day (courses of 3 days with interruptions), children up to a year - 2-5 mg, 1-3 years - 6 mg, 4-5 years - 8 mg, 6-10 years - 10 mg.

Also recommended by hematologists vitamins include B9 and B12. Tablets of folic acid (vitamin B9) should be taken orally after meals - 1-2 mg to twice a day. The daily dose should not exceed 5 mg, the duration of continuous reception - no more than a month.

How to treat hemophilia, for more details, see the publication of Hemophilia. Also see - Villebranch disease treatment.

Alternative treatment of bleeding disorders

With this pathology, alternative treatment can not in any way affect the causes of its occurrence or make "corrections" with the synthesis of plasma and platelet coagulation factors.

Therefore, it is possible to use herbal treatment, which is known for its haemostatic properties: a yarrow, a shepherd's bag, nettle (leaves), water pepper (peppermint), cukushkin goritsvet. Water pepper and shepherd's bag contains vitamin K. For broth, take a tablespoon of dry herbs for a glass of boiling water and cook for 8-10 minutes, it is insisted and accepted: water pepper - a tablespoon 3-4 times a day; decoction of shepherd's bags - one third of a glass three times a day.

It is known for the content of vitamin K and nettle; the water infusion of the leaves of this medicinal plant is prepared by pouring a tablespoon of dry raw material 200 ml of steep boiling water and infusion for 1-1.5 hours (in a closed container). Accepted infusion with bleeding gums, profuse menstrual, nasal bleeding - before eating one tablespoon 3-4 times during the day.

Of all the measures that are usually proposed to prevent the development of certain diseases, virtually none can have a positive effect in the event of a clotting disorder. An exception is the prevention of vitamin K deficiency in the body, as well as the rejection of long-term use of drugs (anticoagulants, aspirin, NSAIDs) that can reduce blood coagulation. And people with poor coagulability are important to avoid situations that can lead to injury and cause bleeding.

The considered disorders of blood coagulability refer to blood diseases. In domestic medicine, there is the concept of "hemorrhagic diseases", as well as "hemorrhagic syndrome," that is, a set of symptoms characterizing the conditions under which hemorrhages occur. And experts of the American Society of Hematology (ASH) allocate only hemorrhagic syndrome of newborns, associated with a lack of vitamin K (code P53 for ICD-10). Obviously, the presence of terminological discrepancies is also characteristic of such a field of clinical medicine as hematology.

[19], [20], [21], [22]