Coagulopathy

The body has a natural process to protect itself when it is cut or injured. Special cells called platelets and proteins in the blood plasma called clotting factors combine to form a substance called fibrin that forms clots and stops the bleeding. Coagulopathies occur when this process is dysfunctional. Also known as bleeding disorders, these conditions have a wide range of possible causes and symptoms.

Definition & Facts

When bleeding occurs, coagulation factors are activated in a specific sequence (coagulation cascade) that help to form a clot. There must be an adequate amount of each coagulation factor and each must be functioning properly in order for normal clotting to occur.

Coagulopathy refers to any disorder of the body’s system of blood clotting or coagulation. This includes hypercoagulable states, when blood coagulates too quickly and may result in blood clots forming in inappropriate parts of the body. A blood clot that forms inside a blood vessel may be called an embolus or thrombus. Bleeding that continues longer than normal is referred to as a hemorrhage.

Symptoms & Complaints

Untreated excessive bleeding can result in damage to the joints, eyes, internal organs, and may be life-threatening. For coagulopathies that cause excessive clotting, a patient may have a history of clotting in unusual places (such as the arms, liver, or kidney). They may have a stroke at a young age or frequent miscarriages.

Blood clots that develop in the bloodstream can become lodged in smaller blood vessels and result in stroke, heart attack, deep vein thrombosis (a blood clot in the veins), or pulmonary embolism depending on their location.

Emergency medical care should be sought for serious symptoms including heavy bleeding, blood in the feces, blood in the urine, vision problems like diplopia (double vision), severe headache or neck pain, continuous vomiting, difficulty walking or standing, or seizures. Additionally, medical care should be obtained for mild, but persistent, external bleeding or joint swelling and joint stiffness.

Causes

Some bleeding disorders may be congenital (that is, present from birth) or inherited. These include hemophilia, von Willebrand disease, and some other clotting factor abnormalities. In these disorders, specific clotting factors are missing or present at insufficient levels.

Conditions that develop later in life and can result in coagulopathies include cancer, liver disease, pregnancy, HIV/AIDS, prolonged immobility, and recent surgery or injury. In addition, nephrotic syndrome, which is a kidney disease in which there is too much protein in the urine (proteinuria) may cause clotting problems. A deficiency in vitamin K can result in prolonged bleeding times.

Some medications or supplements may also affect blood clotting. Hormonal birth control and some types of chemotherapy are known to increase clotting. Medications such as aspirin can thin the blood, resulting in less clotting.

Diagnosis & Tests

Careful evaluation of a patient’s medical history, family history, current symptoms, and laboratory testing are needed to diagnose a coagulopathy. The patient may be referred to a specialist in blood disorders called a hematologist. Genetic testing may identify inherited coagulopathies if they are suspected.

The majority of the diagnostic work-up of a potential coagulopathy will consist of blood tests. Common tests include a CBC (complete blood count), PT (prothrombin time), and aPTT (activated partial thromboplastin time). These tests will help determine if the components of blood are present in the proper proportions and functioning properly. Some of these tests may also be used later to monitor the effects of treatment.

An international normalized ratio (INR) calculation is based on the PT and is used to monitor patients receiving outpatient anticoagulation therapy. If an inappropriate clot is thought to already be present, a test known as a D-dimer may help detect it and determine if treatment or further testing is necessary.

Treatment & Therapy

Depending on the cause of the coagulopathy and the severity of the patient’s presenting symptoms, treatment may include medications to thin the blood (anticoagulants) or break down blood clots that have already formed (thrombolytics), transfusions of blood products, or replacement of the missing clotting factors.

Medication therapy can regulate the body’s ability to form clots. In the hospital, these medicines may be given intravenously or by injection. Therapy can continue at home, either with anticoagulant pills or through training to self-administer injections. For patients on long-term anticoagulant therapy, regular laboratory testing to monitor clotting times is necessary to prevent hemorrhage. Nutritional counseling and consistent dietary levels of vitamin K may help to maintain appropriate clotting times.

Transfusions of blood or blood products may be given to replenish the body’s blood volumes if hemorrhage occurs. In factor replacement therapy, the insufficient or absent clotting factors are replaced with proteins derived from human blood or man-made in a laboratory. Replacement may be given either to treat current bleeding or to prevent bleeding from occurring. Most coagulopathies can be controlled through appropriate medical management, although some will depend on the successful treatment of the underlying disease. 

Prevention & Prophylaxis

Smoking damages the lining of the blood vessels and can cause platelets to clump together, increasing the risk of clotting. Dehydration causes blood vessels to narrow and blood to thicken, raising the risk for blood clots.

Obesity, diabetes, high blood pressure, elevated cholesterol levels, and an irregular heartbeat (e.g. atrial fibrillation) can increase the risk of inappropriate clotting. Managing these conditions and maintaining a healthy weight through diet and exercise can reduce the risk of developing a coagulopathy.