Disseminated intravascular coagulopathy is a clotting disorder and a life-threatening condition associated with bleeding tendencies. Disease triggers are diverse and range from trauma to carcinoma. Prognosis and therapy depend on the underlying disease.
What is disseminated intravascular coagulopathy?
The intrinsic system consists of platelets, vascular endothelium, external vascular tissue, and clotting factors. The system is activated by injury and initiates blood clotting. This results in the so-called coagulation cascade, which results in a plug of fibrin and closes the wound. Coagulopathies are disorders of the intrinsic system. They are divided into minus coagulopathies with increased bleeding tendency and plus coagulopathies with tendency to thrombus formation. Disseminated intravascular coagulopathy is also a coagulopathy. This is a potentially life-threatening condition with bleeding tendency. In this condition, excessive blood clotting occurs in the vascular system, depleting important clotting factors. These clotting factors are no longer available, or only insufficiently available, for blood clotting in the event of injury due to consumption in the vascular system. This form of coagulopathy is an acquired condition from the group of vasculopathies. These are non-inflammatory vascular diseases with vascular occlusion. Synonymous with the term disseminated intravascular coagulopathy are the medical terms consumption coagulopathy and defibrination syndrome. The disease consists of three phases: a pathologic activation of the coagulation system, a recognizable deficit regarding coagulation potential, and a defibrination phase.
Causes
Consumptive coagulopathy usually develops in the setting of severe underlying disease. Three groups of trigger mechanisms are distinguished: In addition to rapid wash-in of prothrombin activators, causative factors may include massively active plasmatic coagulation by an endogenous pathway or mediator-mediated coagulation activation. This makes, for example, shock resulting from trauma, hypovolemia, and gram-negative sepsis conceivable causes of disseminated intravascular coagulopathy. Snake venom or complications of childbirth may also be causative. Among the most important causes from the field of birth complications are premature detachment of the placenta and amniotic fluid embolism. Equally causative of consumptive coagulopathy may be surgical procedures on the prostate, pancreas, or lung that are associated with increased release of prothrombin activators. Consumptive coagulopathies may also occur in the setting of Waterhouse-Friderichsen syndrome. This is due to gram-negative endotoxin exposure associated with the disease. In addition, infections may be a trigger for disseminated intravascular coagulopathy. Massive hemolysis can also occur in transfusion incidents, which can just as easily result in consumptive coagulopathy. Malignancies may also be considered as a setting for coagulopathy. The phenomenon has been observed particularly frequently after rapid malignancy decay. In addition, during surgery using a heart–lung machine, extracorporeal circulation can cause disseminated intravascular coagulopathy.
Symptoms, complaints, and signs
In the initial stage of pathologic activation, no deviation from the healthy norm can yet be discerned in patients with consumptive coagulopathy. The pathologic process is nonetheless already under way. TFPI and antithrombin are consumed in the vascular system. The procoagulant effect of different components of the coagulation cascade initiates disseminated intravascular coagulopathy. Pathologically high levels of neurotransmitters are present in the body. In addition to histamine and serotonin, the elevation may involve adrenaline, for example. Small blood clots form in the blood capillaries, arterioles and venules. Blood vessels in the lungs, kidneys and heart become clogged. Liver function may also be affected. In the second stage of the disease, platelets, clotting factors and inhibitors drop sharply as they are consumed intravascularly. As a result, fibrinolysis occurs. The levels of fibrin cleavage product are increased and the level of fibrin drops.Undirected clotting in the vessels consumes blood components that are necessary for blood clotting. Deficiencies occur especially with regard to platelets, fibrinogens and clotting factors. The organism is now no longer able to close damaged blood vessels itself and a hemorrhagic diathesis occurs. This is followed by the defibrination stage. In addition to platelets and coagulation factors, antithrombin is now also reduced. The deficiency symptoms are clinically expressed inform of shock associated with multi-organ failure due to thrombi or bleeding tendency. Spontaneous bleeding without injury is seen on the skin and in various organs.
Diagnosis and course
The diagnosis of consumptive coagulopathy is made on the basis of laboratory parameters such as D-dimer, platelet count, and the Quick value. In addition to thrombocytopenia, there is a prolongation of the PTT, a decrease in the Quick value, and a decrease in antithrombin III. Furthermore, consumption of fibrinogen can be observed, which is accompanied by proteolytic activation or degradation of other coagulation factors. Prognosis depends critically on the cause, therapeutic options, and complications. In the presence of associated manifestations such as renal failure, the prognosis tends to be unfavorable.
Complications
In the rules, the disease is a relatively dangerous condition for the patient, which must be treated in any case. Without such, death may occur in the worst case. Blood clots are formed, which can clog the blood vessels in the further course of the disease. This results in reduced blood flow to the heart and kidneys. In most cases, the body itself can no longer close the damaged blood vessels, which can lead to a life-threatening condition. Individual organs may fail and the patient may die. Diagnosis of the disease is relatively simple and clear, so treatment can be started quickly. As a rule, treatment is carried out with the help of drugs and blood transfusions. This results in a positive course of the disease. However, a relatively long stay in hospital is necessary for the patient, and the quality of life is greatly reduced. Even after recovery, the patient must take it easy and not engage in heavy physical activities or sports. If treatment is successful, life expectancy is usually not reduced.
When should you see a doctor?
If signs of bleeding tendency occur after shock, extensive burns, trauma, or severe sepsis, a consumptive coagulopathy may be present. This condition, also known as disseminated intravascular coagulopathy, also occurs in obstetric complications, preeclampsia, eclampsia, and amniotic fluid embolism. So do sepsis and complications of surgical procedures on the heart, lungs and internal organs. In most cases, the attending physician will self-diagnose the consumptive coagulopathy and initiate the necessary medical measures. In the chronic form, those affected can sometimes detect disseminated intravascular coagulopathy on their own based on symptoms. Patients with cirrhosis of the liver, cardiac defects, metastatic carcinoma, or hemoblastosis should consult a physician if there is increased bleeding or signs of bleeding anemia. Because disseminated intravascular coagulopathy is almost always life-threatening, emergency medical attention must always be sought. Patients who are already receiving treatment should inform the nursing staff or physician if they suspect anything. The latter will perform an examination and, if necessary, initiate treatment directly.
Treatment and therapy
Manifest but early consumption coagulopathy is associated with reversible dysfunction of the liver, kidneys, and lungs. At this stage, the main treatment options include heparinization. If the platelet count drops threateningly, thrombosis and bleeding tendency must be considered as treatment complications. Heparinization should be used only moderately in this case. In severe consumption coagulopathy in later stages, shock reactions set in at organs such as the lungs or kidneys. The hemoglobin concentration decreases. Fragmentocytes form until there is an almost absolute lack of fibrinogen. Heparinization is moderated during this phase.An antithrombin III concentrate is administered if bleeding occurs. If Quick values are below 30 percent, PPSB is administered. Fibrinogen concentrates may also be administered if concentrations are 0.1 g/l or higher. Anemias are treated by blood transfusion at this stage. Fresh frozen plasma and factor concentrates are a final and relatively controversial treatment option. Among the rather new therapeutic approaches is the administration of recombinant activated protein C, which is active as a coagulation inhibitor. However, the first preparations from this field had to be withdrawn from the market due to bleeding complications. The future may bring applicable preparations in this field. In some countries, antifibrinolytics are given in cases of consumption coagulopathy. However, this step is considered controversial in Germany.
Outlook and prognosis
Disseminated intravascular coagulopathy represents a life-threatening condition that can be acquired in the setting of various underlying diseases. In this regard, the prognosis of this disorder depends on the underlying cause. Fatal courses result both from increased thrombus formation and from an increased bleeding tendency in the later stages of the disease. In the first stage, microthrombi initially form and either remain asymptomatic or already cause damage to various organs supplied by arterioles, venules, or blood capillaries. These include the heart, kidneys, lungs, liver or adrenal glands. This stage can still be well controlled by treatment with heparin, because this reduces blood clotting, but there are still enough platelets. If, in a second stage, the number of platelets is greatly reduced due to their high consumption, an increased bleeding tendency is added. The use of heparin is contraindicated here, as this only increases the bleeding tendency. Here, the prognosis depends mainly on the treatment options for the underlying diseases. The third stage of disseminated intravascular coagulopathy is characterized by full-blown shock. In addition to multiorgan failure due to emboli, there is increased bleeding. Spontaneous bleeding occurs in various organs. In this phase, patient survival depends on therapeutic control of underlying diseases and complications.
Prevention
Disseminated intravascular coagulopathy and its complications can be prevented by physicians by daily monitoring of coagulation levels in patients at risk. Patients with risk factors receive heparin for prevention.
Follow-up
In most cases of this disease, no direct measures or options for follow-up care are available to the affected person. In this case, the affected person is first dependent on a comprehensive diagnosis and treatment of this disease, otherwise death or other serious complications may occur. Therefore, the main priority in this disease is an early diagnosis with early treatment, since it is also not possible for the disease to heal itself. The further course depends very much on the exact underlying disease, which of course must be treated and avoided. The treatment itself is carried out with the help of medication and blood transfusions. The affected person is dependent on regular transfusions to alleviate the symptoms. When taking the medication, it should be noted that it should be taken regularly and the correct dosage should be observed. If there are any questions or uncertainties, a doctor should always be consulted first. The support and loving care of friends and one’s own family is also very important in this disease, so that the treatment is accelerated. However, in many cases, the life expectancy of the patient is significantly reduced by this disease.
This is what you can do yourself
Disseminated intravascular coagulopathy occurs in people who are normally already receiving medical treatment for a serious illness. The organism is already weakened and medical care is taking place. Due to the circumstances, the possibilities of self-help are focused on improving the well-being in the situation, since a cure by one’s own efforts is unlikely. To follow the instructions of the doctors or the nursing staff. Changing the treatment plan on one’s own responsibility is not recommended, as complications may occur.A fundamentally positive attitude to life helps the person affected to cope better with the adversities caused by the disease. The zest for life should also be strengthened during this time as far as possible. Talking to people you trust or exchanging ideas with other sufferers can help to talk about emotional burdens and reduce fears. If professional help is needed, a psychologist should be consulted. The diet should be balanced and healthy so that the body’s defense system receives sufficient nutrients and vitamins. Food intake has a crucial part in nourishing the organs and stabilizing the organism. Therefore, the consumption of healthy food is of elementary importance even in the case of complaints such as lack of appetite.
