Hypercoagulability is a pathologically 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?
Thrombosis often goes unnoticed. Even severe thrombosis, which later leads to a pulmonary embolism, is often initially symptomless. See AbbreviationFinder for abbreviations related to Hypercoagulability.
Blood clots faster in patients with hypercoagulability than in healthy people. The increased coagulability is caused by an increase in coagulation-promoting factors or a reduction in anticoagulant factors.
The consequences of such a coagulation disorder are spontaneously occurring thromboses. Thromboses are vascular occlusions caused by clots. Thrombosis in the coronary arteries can trigger a heart attack, thrombosis 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 known as congenital hypercoagulability. It can have various causes. The coagulation disorder can be inherited in an autosomal dominant manner. Genetic errors can lead to a lack of protein C, protein S or antithrombin III.
A deficiency in these enzymes results in reduced inactivation of the coagulation cascade and hypofibrinolysis. As a result, blood clots form more quickly and these blood clots are broken down more slowly. 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.
The activated protein C inhibits blood clotting. Patients with APC resistance develop venous thrombosis more quickly than healthy people. Congenital homocysteinemia is also associated with hypercoagulability. Acquired hypercoagulability is usually due to impaired liver function. Reduced synthesis of protein S, protein C and antithrombin III develops.
The reduced synthesis can be caused by a lack of vitamin K, by outflow disorders or by synthesis limitations in the context of liver insufficiency. Liver failure 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. Antiphospholipid antibodies circulate in the blood.
Among other things, these are directed against coagulation factors and cause an increased willingness to coagulate. Antiphospholipid syndrome can also be triggered by rheumatoid arthritis, HIV, hepatitis B, malaria, or drugs such as chlorpromazine or propranolol. Hypercoagulability can also occur in the context of heparin-induced thrombocytopenia. It is caused by adhesion of the platelets.
Hypercoagulability also occurs in the context of consumption coagulopathy. Here, the increased coagulability causes a consumption of coagulation factors. As a result, there is a lack of coagulation factors and thus an increased tendency to bleed.
In hypercoagulability, blood coagulation is increased. This results in spontaneous thrombosis, which can also recur. Hypercoagulability is part of the Virchow triad. Accordingly, three factors are particularly relevant for the development of thrombosis:
- changes in the vessel wall
- changes in flow velocity
- Changes in blood composition (hypercoagulability)
Symptoms, Ailments & Signs
The symptoms of a thrombosis can vary greatly depending on the location and extent of the vascular occlusion. Thrombosis often goes unnoticed. Even severe thrombosis, which later leads to a pulmonary embolism, is often initially symptomless. In the case of a pronounced leg vein thrombosis, however, there is typically swelling and a feeling of warmth in the lower leg or in the entire leg.
The swelling often causes a feeling of tightness. Elevating the affected extremity brings relief. The skin in the affected area is red and tight. A blue color may also be visible. The most dangerous complication of deep vein thrombosis is pulmonary embolism. The thrombus detaches, migrates into the lungs and occludes important vessels there.
The pulmonary embolism can be fatal. Postthrombotic syndrome can occur as a late complication after deep vein thrombosis. If thrombi form in the coronary arteries due to hypercoagulability, a heart attack occurs. The typical symptoms of a heart attack are intense pressure in the heart area, pain in the chest radiating to the arms or upper abdomen, tightness, nausea, vomiting and shortness of breath.
However, clot formation in a cerebral artery leads to an ischemic apoplexy. This condition is also known as a stroke. The circulatory disturbance leads to a lack of oxygen and thus to the death of brain tissue.
Diagnosis & course of disease
The diagnosis is made in the laboratory. For this purpose, protein C, protein S and antithrombin III are determined. One or more of these values are reduced in hypercoagulability. If a factor V Leiden mutation is suspected, a molecular genetic test is carried out.
Complications
In many cases, hypercoagulability goes unnoticed, making early treatment of this disease impossible. The affected person can suffer a pulmonary embolism due to the hypercoagulability and, in the worst case, die from it. As a rule, swelling primarily spreads to the lower leg, which can later cover the entire leg. There is also pain and discoloration of the skin on the legs.
This leads to movement restrictions and thus to severe impairments in everyday life. Hypercoagulability can also later lead to a heart attack, which is preceded by chest pain. These can also spread to other areas of the body, causing pain in the neck and back. The patient also suffers from shortness of breath, vomiting and nausea.
A heart attack can also be fatal for the patient. The treatment of hypercoagulability takes place causally and is usually carried out with the help of drugs. There are no further complications. However, complete healing cannot be guaranteed. The further course of the disease depends heavily on the severity.
When should you go to the doctor?
Consultation of a doctor is necessary as soon as circulatory disorders appear. 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 for a long time or if they continuously increase in intensity, there is cause for concern. In the case of swelling, restricted mobility or a loss of the usual level of performance, the observations should be discussed with a doctor. If there is a feeling of tightness in the chest, changes in the complexion or discoloration, a doctor’s visit is advisable.
A blue color in particular is considered a warning. If the person affected suffers from nausea, vomiting or dizziness, it is advisable to clarify the symptoms. In the event of shortness of breath or interruptions in breathing, a doctor’s visit should be initiated as soon as possible. Loss of consciousness or sudden collapse requires immediate medical attention. An ambulance must be called and their instructions must be followed to ensure the patient’s survival. Chest pain indicates an emergency. If the pain extends to the arm, shoulder or upper abdomen, a doctor is required. If you experience breathing difficulties, sleep disturbances, general malaise or behavioral problems, you should consult a doctor.
Treatment & Therapy
Therapy for hypercoagulability depends on the cause. If several spontaneous thromboses have already occurred, the congenital hypercoagulability must be treated with medication for life. So-called anticoagulants are used for this. These blood thinners inhibit blood clotting. Known anticoagulants are coumarin derivatives such as phenprocoumon or warfarin.
Heparin cannot be used, since the lack of antithrombin III reduces the effectiveness of the drug. The therapy of acquired hypercoagulability is also dependent on the cause. In the case of a vitamin K deficiency, vitamin K is substituted. It may also be necessary to carry out thrombosis prophylactic heparinization.
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 carried out permanently.
Outlook & Forecast
If left untreated, hypercoagulability has an unfavorable prognosis. The effects of the disorder can trigger various life-threatening conditions in the sufferer. An acute situation often arises in which the patient dies within a few minutes. If hypercoagulability is noticed and diagnosed in good time, the life-threatening consequences can be significantly minimized in long-term therapy. The causative disease must be found and treated in order to have a good prognosis.
Not all underlying diseases of the blood clotting disorder can be cured. In many cases, the living conditions have to 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 the organ tissue, there may be a need for a donor organ to ensure the patient’s life. A surgical procedure is always associated with various risks and complications. If the operation is successful and the donor organ is well accepted by the organism, there will be significant improvements in health.
Although limitations remain, a good quality of life can be achieved under changed conditions. Nevertheless, the vital and blood values must be checked regularly throughout life. In the 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, if the therapy plan is followed, a favorable prognosis is given.
Prevention
Congenital hypercoagulability is innate and therefore cannot be prevented. Acquired hypercoagulability can at best be prevented by early detection and treatment of the underlying disease.
Aftercare
In most cases, patients with hypercoagulability have no or only very few follow-up measures and options available. It is particularly important to ensure early diagnosis and subsequent treatment in order to properly alleviate the symptoms and prevent further deterioration of the symptoms. The earlier the disease is detected, the better the further course of the disease is usually.
This disease is treated in most cases by taking medicines. The affected person is dependent on the right dosage with regular intake in order to relieve the symptoms properly and, above all, permanently. The doctor’s instructions must always be followed. Regular examinations should also be carried out by a doctor in order to control the situation.
Since hypercoagulability is a congenital disease, it cannot be completely cured either. If the person concerned wants to have children, genetic counseling can also be carried out to prevent the disease from reoccurring. Despite treatment, the disease often leads to a reduced life expectancy for the patient.
You can do that yourself
In most cases, hypercoagulability is treated with medication. Those affected should ensure that they take blood thinners regularly to avoid thrombosis and other complications.
Furthermore, the disease can also lead to a lack 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, for example to prevent a heart attack. 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 life. Above all, the help of friends and family proves to be positive. Talking to a psychologist or to close friends and relatives about the disease can also alleviate depression.
In the case of children, direct education should take place in order to show the correct course of the disease. Further treatment depends heavily on the underlying disease, so that no general prediction can be made here.