Hypercoagulability refers to an abnormally increased coagulability of the blood. It is associated with a tendency to form thrombi and is considered one of the main risk factors for phlebothrombosis.
What is hypercoagulability?
In patients with hypercoagulability, the blood clots more quickly than in healthy people. The increased clottability is caused by an increase in factors that promote clotting or a decrease in factors that inhibit clotting. The consequence of such a coagulation disorder is spontaneously occurring thromboses. Thromboses are vascular occlusions caused by clots. Thromboses in the coronary vessels can trigger a heart attack, thrombi in the vessels supplying the brain lead to a stroke. Therapy for hypercoagulability depends on the cause.
Causes
Hypercoagulability can be acquired or congenital. Congenital hypercoagulability is also called congenital hypercoagulability. It may have several underlying causes. The coagulation disorder can be inherited in an autosomal-dominant manner. Genetic defects can lead to a deficiency of protein-C, protein-S or antithrombin III. Deficiency of these enzymes results in reduced inactivation of the coagulation cascade and hypofibrinolysis. As a result, blood clots form more rapidly on the one hand and are degraded more slowly on the other. A factor V mutation as APC resistance can also be inherited in an autosomal dominant manner. APC resistance is characterized by a weak response to activated protein C. Activated protein C inhibits blood clotting. Patients with APC resistance suffer venous thrombosis more rapidly than healthy individuals. Congenital homocysteinemia is also associated with hypercoagulability. Acquired hypercoagulability is usually due to a functional impairment of the liver. In this case, a reduced synthesis of protein S, protein C and antithrombin III develops. The reduced synthesis can be caused by a deficiency of vitamin K, by outflow disorders or by synthesis restrictions in the context of liver insufficiencies. Liver insufficiency is usually the result of liver cirrhosis. Autoimmune diseases can also cause hypercoagulability. An example of such an immunocoagulopathy is the antiphospholipid syndrome of systemic lupus erythematosus. In this case, antiphospholipid antibodies circulate in the blood. These are directed, among other things, against clotting factors and cause an increased tendency to clot. Antiphospholipid syndrome can also be triggered by rheumatoid arthritis, HIV, hepatitis B, malaria or drugs such as chlorpromazine or propranolol. Hypercoagulability may also occur in the setting of heparin-induced thrombocytopenia. It is caused by adhesion of platelets. Hypercoagulability also occurs in the context of a consumption coagulopathy. Here, increased coagulability causes consumption of clotting factors. As a result, there is a deficiency of clotting factors and thus an increased tendency to bleed. In hypercoagulability, blood clotting is increased. This causes spontaneous thrombosis, which can also recur. Hypercoagulability is part of the Virchow triad. According to this, three factors in particular are relevant to the development of thrombosis:
- Changes in the vessel wall
- Changes in the flow velocity
- Changes in blood composition (hypercoagulability).
Symptoms, complaints and signs
Symptoms of thrombosis can vary greatly depending on the location and on the extent of the vessel occlusion. Frequently, thromboses go unnoticed. Even severe thromboses that lead to pulmonary embolism at a later stage are often asymptomatic at first. In the case of a pronounced leg vein thrombosis, however, there is typically swelling and a feeling of warmth in the lower leg or the entire leg. The swelling often causes a feeling of tightness. Elevation of the affected extremity brings relief. The skin in the affected area is red and taut. A blue discoloration may also be visible. The most dangerous complication of deep vein thrombosis is pulmonary embolism. In this case, the thrombus breaks loose, travels to the lungs and blocks important vessels there. Pulmonary embolism can be fatal. A late complication that can occur after deep vein thrombosis is post-thrombotic syndrome.If thrombi form in the coronary vessels due to hypercoagulability, a heart attack occurs. The typical symptoms of a heart attack are intense pressure in the heart area, chest pain radiating to the arms or upper abdomen, a feeling of tightness, nausea, vomiting and shortness of breath. Clot formation in a cerebral artery, on the other hand, leads to ischemic apoplexy. This condition is also known as a stroke. In this case, the circulatory disturbance leads to a lack of oxygen and thus to the death of brain tissue.
Diagnosis and course of the disease
Diagnosis is made in the laboratory. For this purpose, protein C, protein S, and antithrombin III are determined. One or more of these levels are decreased in hypercoagulability. If a factor V Leiden mutation is suspected, molecular genetic testing is performed.
Complications
In many cases, hypercoagulability goes unnoticed, so early treatment of this condition is not possible. The affected person may suffer a pulmonary embolism due to hypercoagulability and, in the worst case, die from it. As a rule, swelling spreads primarily to the lower leg, which can later cover the entire leg. Furthermore, there is also pain on the legs and a coloring of the skin. Movement is restricted and this leads to severe impairments in everyday life. Hypercoagulability can also later lead to a heart attack, which is preceded by chest pain. This can also spread to other areas of the body, leading to pain in the neck and back. The patient also suffers from shortness of breath, vomiting and nausea. The heart attack can also be fatal for the patient. The treatment of hypercoagulability is causal and is usually carried out with the help of medication. No further complications occur. However, a complete cure cannot be guaranteed. The further course of the disease depends largely on its manifestation.
When should you go to the doctor?
Consultation with a doctor is necessary as soon as disturbances in blood circulation occur. If there is an unusual sensation of warmth in the body or cold limbs, the signs should be examined and treated. If the symptoms persist over a longer period of time or continuously increase in intensity, there is reason for concern. If there is swelling, restriction of movement, or a loss of the usual level of performance, observations should be discussed with a physician. If there is a feeling of tightness in the chest, changes in the appearance of the skin or discoloration, a visit to the doctor is advisable. Blue discoloration in particular is considered a warning sign. If the affected person suffers from nausea, vomiting or dizziness, a clarification of the symptoms is recommended. In case of shortness of breath or intermittent breathing, a visit to the doctor should be initiated as soon as possible. Unconsciousness or sudden collapse requires immediate medical attention. An emergency physician must be called and his or her instructions followed to ensure the patient’s survival. Chest pain indicates an emergency. If the pain moves to the arm, shoulder or upper abdomen, a physician is needed. If breathing difficulties, sleep disturbances, a general malaise or behavioral problems occur, a physician should be consulted.
Treatment and therapy
Therapy for hypercoagulability depends on the cause. If multiple spontaneous thromboses have already occurred, congenital hypercoagulability must be treated with medication for life. So-called anticoagulants are used for this purpose. These blood thinners inhibit blood clotting. Known anticoagulants are coumarin derivatives such as phenprocoumon or warfarin. Heparin cannot be used because the drug’s effectiveness is reduced by the lack of antithrombin-III. Therapy for acquired hypercoagulability is also cause-dependent. In the case of vitamin K deficiency, vitamin K is substituted. Thromboprophylactic heparinization may also be required. If the hypercoagulability is due to liver disease, this causative disease must be treated. Antithrombin III deficiency caused by liver disease can be treated in the short term by antithrombin III substitution. However, this therapy cannot be sustained.
Prospect and prognosis
If left untreated, hypercoagulability has an unfavorable prognosis.The effects of the disorder can trigger various life-threatening conditions in the affected person. Often an acute situation occurs in which the patient dies within a few minutes. If hypercoagulability is noticed and diagnosed in time, the life-threatening consequences can be significantly minimized in long-term therapy. The causative disease must be found and treated to ensure a good prognosis. Not all underlying diseases of the blood coagulation disorder are curable. In many cases, living conditions must be changed and optimized in addition to drug treatment. If organ diseases are present, they must be evaluated individually. In the case of irreparable damage to organ tissue, there may be a need for a donor organ in order to secure the patient’s life. An operation is always associated with various risks and complications. If the operation is successful and the donor organ is well accepted by the organism, significant improvements in health occur. Although limitations remain, a good quality of life can be achieved under changed conditions. Nevertheless, regular monitoring of vital and blood values must be carried out throughout the patient’s life. In case of changes and abnormalities, new treatments are necessary. Overall, blood coagulation does not lead to freedom from symptoms in a large number of patients. Nevertheless, a favorable prognosis is given if the treatment plan is followed.
Prevention
Congenital hypercoagulability is congenital and thus cannot be prevented. At best, acquired hypercoagulability can be prevented by early recognition and therapy of the underlying disease.
Follow-up
In most cases of hypercoagulability, the patient has no or very few measures and options for aftercare. In this case, the most important thing is early diagnosis with subsequent treatment to properly alleviate the symptoms and prevent further worsening of the symptoms. The earlier the disease is detected, the better is usually the further course of the disease. In most cases, this disease is treated by taking medication. In doing so, the affected person is dependent on a correct dosage with regular intake in order to alleviate the complaints correctly and, above all, permanently. The doctor’s instructions must always be followed. Regular examinations by a doctor should also be carried out to monitor the situation. Since hypercoagulability is a congenital disease, it cannot be completely cured. If the affected person wishes to have children, genetic counseling can also be performed to prevent the recurrence of this disease. However, it is not uncommon for the disease to result in a reduced life expectancy for the patient despite treatment.
What you can do yourself
In most cases, hypercoagulability is treated by taking medication. In doing so, those affected should make sure to take blood thinners regularly to avoid thrombosis and other complications. Furthermore, the disease can also lead to a deficiency of vitamin K, so that this vitamin must be taken through supplements. Older people in particular should take part in regular check-ups with various specialists in order to prevent a heart attack, for example. Even slight pain in the respective areas can indicate the disease. Since many of those affected suffer from restricted mobility due to hypercoagulability, they are dependent on the help of other people in their everyday lives. In this context, the help of friends and family proves to be particularly beneficial. Discussions with psychologists or with the closest friends and relatives about the disease can also alleviate depression. In the case of children, direct education should take place in order to show a proper course of the disease. The further treatment depends strongly on the underlying disease, so that no general prediction can be made here.
