Blood clotting disorder

Disruptions in the functioning of the body's most important system - the hemostasis system, designed to protect a person from blood loss - manifest themselves as a violation of blood clotting, that is, pathologies of the coagulation of protein components of the blood in the event of bleeding.

The ability of blood to clot can be reduced for various reasons, leading to severe and life-threatening coagulopathies.

Causes clotting disorders

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

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

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Pathogenesis

The pathogenesis of deviations in the hemocoagulation process is directly related to the imbalance of blood coagulation factors, since only when they are activated does the natural mechanism of blood clot formation and bleeding stop occur. An equally important factor is the number of platelets in the blood, synthesized by bone marrow cells.

The existing classification of blood clotting disorders divides them – depending on the etiology – into acquired, genetically determined 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 conditions, during surgical interventions, after massive blood transfusions, during septicemia and severe infectious diseases, during the disintegration of large malignant neoplasms;
• vitamin K deficiency (due to bile duct obstruction or poor bowel function);
• pernicious (megaloblastic) anemia, which occurs due to a deficiency of cyanocobalamin (vitamin B12) and folic acid (vitamin B9); this pathology can be a consequence of severe dysbacteriosis, as well as diphyllobothriasis (parasitism in the body by the broad tapeworm);
• tumor diseases of the hematopoietic system (leukemia, hemoblastosis) with damage to bone marrow stem cells;
• side effects of anticoagulant drugs used in the treatment of thrombosis, as well as cytostatics used in cancer chemotherapy.

Read also - Acquired platelet dysfunctions

Causes of blood clotting disorders include the following genetic and congenital pathologies:

• hemophilia A (deficiency of antihemophilic globulin – clotting factor VIII), hemophilia B (deficiency of clotting factor IX) and hemophilia C (deficiency of thromboplastin – factor XI);
• von Willebrand disease (constitutional thrombopathy or von Willebrand-Jurgens syndrome, when there is a lack of antihemophilic globulin in the blood);
• thrombocytopenic purpura (Werlhof's disease);
• Glanzmann's hereditary thrombasthenia;
• congenital afibrinogenemia (absence of fibrinogen in the blood) and dysfibrinogenemia (structural defects of fibrinogen molecules).

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

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Symptoms clotting disorders

Typical symptoms of blood clotting disorders in almost all of the above pathologies appear in various combinations and with varying intensity.

The first signs include hemorrhagic syndrome - a tendency to develop hemorrhages into the skin and subcutaneous tissue, as well as increased bleeding of the mucous membranes.

Bleeding can be capillary, hematoma or mixed. Thus, in case of deficiency of such blood coagulation factors as prothrombin, proaccelerin, proconvertin, Stewart-Prower factor (in von Willebrand disease, thrombocytopenia or dysfibrinogenemia), small red-violet spots - petechiae (capillary hemorrhages) - appear on the skin of the legs in the ankle area and on the feet.

If there is a deficiency of antihemophilic globulin, bruises (ecchymoses) constantly appear. Subcutaneous hematomas are characteristic of hemophilia, as well as of most acquired blood clotting disorders, including after long-term use of anticoagulant drugs.

In addition, symptoms of blood clotting disorders are expressed in frequent spontaneous nosebleeds, bleeding gums, significant blood loss during menstruation (menorrhagia), prolonged bleeding even with minor damage to the smallest vessels. Frequent reddening of the whites of the eyes is possible, melena (black stool) may also be observed, which indicates bleeding localized in the gastrointestinal tract. In hemophilia, blood flows not only into the tissues of the abdominal organs and muscles, but also into the joints (hemarthrosis). This causes necrosis of bone tissue, a decrease in the calcium content in it, and subsequent functional complications of the musculoskeletal system.

For symptoms of thrombohemorrhagic syndrome and its possible consequences (which can be fatal), see Disseminated intravascular coagulation (DIC) syndrome.

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

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Diagnostics clotting disorders

Clinical diagnosis of blood clotting disorders begins with collecting anamnesis and necessarily includes laboratory tests of the patients' blood.

The following blood tests are required:

• general clinical;
• coagulogram (blood clotting time is determined);
• PTT and PTI (prothrombin time and prothrombin index provide an idea of the speed of the coagulation process);
• TV (determines thrombin time, i.e. the rate of conversion of fibrinogen into fibrin);
• ABC test (determines activated blood clotting time);
• on platelet aggregation with stimulation by adenosine diphosphate (ADP);
• APTT (determines the presence of a deficiency of several plasma coagulation factors at once);
• on antithrombin III (a glycoprotein anticoagulant that inhibits the blood clotting process).

Read more - Hemostasis System Research

Instrumental diagnostics (X-ray, ultrasound, MRI) can be used to determine the condition of the liver, spleen, intestines or brain.

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Differential diagnosis

Considering the polyetiology of blood clotting disorders, only differential diagnostics provides a real opportunity to determine the specific cause of coagulopathy and prescribe the correct therapy.

Treatment clotting disorders

In case of bleeding, treatment of blood clotting disorder requires urgent measures – see Bleeding. Medicines that promote thrombus formation (Fibrinogen, Thrombin) are also used in a hospital setting to stop bleeding.

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

In many cases, the use of antihemorrhagic and hemostatic drugs is recommended: those that activate the formation of thromboplastin, heparin antagonists, fibrinolysis inhibitors, as well as agents that promote thrombus formation.

Medicines prescribed to stimulate the production of tissue thromboplastin (III blood coagulation factor) - Dicyon (other trade name Etamzilat). This drug is used to stop capillary and internal bleeding, is available as an injection solution, and in 0.5 g tablets for the treatment of blood clotting disorders. The dose is determined by the doctor in accordance with blood counts (usually 0.25-0.5 g two or three times a day), the duration of administration also depends on the diagnosis. Dicyon gives side effects in the form of headache, nausea, decreased blood pressure, skin hyperemia and impaired sensitivity of the skin on the legs. The drug is contraindicated if increased bleeding and hematomas are the result of taking anticoagulants.

Medicines such as Aminocaproic acid, Tranexam, Ambene, etc., inhibit the dissolution of a blood clot (fibrinolysis). Aminocaproic acid (Epsilon-aminocaproic acid, Acikapron, Afibrin, Karpacid, Karpamol, Epsamon) in tablets of 0.5 g is prescribed orally based on weight, the maximum daily dose should not exceed 15 g. Side effects include dizziness, catarrhal symptoms, nausea, and diarrhea. Aminocaproic acid is contraindicated in cases of poor kidney function.

The hemostatic agent Tranescam (tranexamic acid) in tablets of 0.25 g is recommended for frequent nosebleeds 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 blood clotting disorders, a drug containing an analogue of vitamin K is often recommended – Vikasol (Menadione sodium bisulfite, Menadione). Vikasol (in tablets of 15 mg) increases the ability of blood to clot, acting on the production of vitamin K-dependent prothrombin and proconvertin (clotting factors). The dosage of this drug: for adults - 15-30 mg per day (in courses of 3 days with breaks), children under one year - 2-5 mg, 1-3 years - 6 mg, 4-5 years - 8 mg, 6-10 years - 10 mg.

Also recommended by hematologists are vitamins B9 and B12. Folic acid tablets (vitamin B9) should be taken orally after meals - 1-2 mg up to two times a day. The daily dose should not exceed 5 mg, the duration of continuous intake - no more than a month.

For more information on how hemophilia is treated, see the publication Hemophilia. Also see – Treatment of von Willebrand disease.

Folk remedies for blood clotting disorders

In this pathology, folk treatment cannot in any way influence the causes of its occurrence or make “corrections” to the synthesis of plasma and platelet blood clotting factors.

Therefore, you can use herbal treatment that is known for its hemostatic properties: yarrow, shepherd's purse, stinging nettle (leaves), water pepper (pepper knotweed), adonis. Water pepper and shepherd's purse contain vitamin K. For the decoction, take a tablespoon of dry grass per glass of boiling water and boil for 8-10 minutes, infuse and take: water pepper - a tablespoon 3-4 times a day; shepherd's purse decoction - a third of a glass three times a day.

Nettle is also known for its vitamin K content; an aqueous infusion of the leaves of this medicinal plant is prepared by pouring 200 ml of boiling water over a tablespoon of dry raw material and infusing for 1-1.5 hours (in a closed container). The infusion is taken for bleeding gums, heavy periods, nosebleeds - before meals, one tablespoon 3-4 times a day.

Of all the measures that are usually offered to prevent the development of certain diseases, almost none can give a positive effect in the case of blood clotting disorders. The exception is the prevention of vitamin K deficiency in the body, as well as the refusal of long-term use of drugs (anticoagulants, aspirin, NSAIDs), which can reduce the level of blood clotting. And for people with poor clotting, it is important to avoid situations that can lead to injury and cause bleeding.

The above-mentioned blood clotting disorders are related to blood diseases. In Russian medicine, there is a concept of "hemorrhagic diseases" and "hemorrhagic syndrome", i.e. a set of symptoms characterizing conditions in which hemorrhages occur. And specialists from the American Society of Hematology (ASH) distinguish only hemorrhagic syndrome of the newborn, associated with a lack of vitamin K (code P53 according to ICD-10). Obviously, the presence of terminological differences is also characteristic of such a field of clinical medicine as hematology.

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