Coagulopathies, also called coagulation disorders, occur when blood does not clot normally.

What Is a Coagulopathy?

In normal coagulation, or blood clot formation, blood cells called platelets create a plug at the site of an injury to stop the bleeding. A protein called fibrin then forms a mesh covering that strengthens and stabilizes the plug. Usually, the clot will dissolve as the injury heals.

Coagulopathy interrupts this complex process, which results in abnormal bleeding or clots that do not disappear as they should.

Causes of Coagulopathies

Blood clotting disorders can be inherited, which means they are passed down by parents. They can also be acquired at some point later in life. The underlying cause may involve a defect in blood vessels, blood platelets or blood clotting factors.

Types of Coagulopathies

The two primary types of coagulopathies involve blood that does not clot enough or blood that clots too much. Insufficient clotting can result in prolonged or excessive bleeding called a hemorrhage. Excessive clotting can cause clots that block the blood vessel where they form, known as thrombosis, or that break up and cause blockage elsewhere, known as embolism. 

Hereditary coagulopathies

Hereditary blood clotting disorders are caused by genetic differences. Some of the most common inherited coagulopathies include:

• Hemophilia, a condition in which the blood lacks sufficient clotting proteins. Hemophilia A is caused by a missing or defective blood clotting factor VIII (FVIII), while hemophilia B is caused by a deficiency of factor IX. Both types of hemophilia cause excessive bleeding externally or internally.
• Inherited thrombocytopenia (ITP), a disorder in which the number of blood platelets is too low, caused by a genetic mutation affecting platelet formation.
• Von Willebrand disease (vWD), a rare disease caused by a defect or deficiency in von Willebrand Factor (VWF). The disease slows down the clotting process, resulting in heavy bleeding or excessive bruising.

Acquired coagulopathies

Acquired blood clotting disorders may occur for a variety of reasons, including disease or environmental factors. Some of the most common risk factors associated with acquired coagulopathies include:

• autoimmune diseases such as lupus
• bone marrow disorders such as aplastic anemia
• certain types of cancer
• exposure to toxic substances
• liver disease
• rare complications of pregnancy such as disseminated intravascular coagulation (DIC)
• reaction to certain medications
• severe infection
• traumatic injury
• vitamin K deficiency

Signs and Symptoms of Coagulopathies

Because coagulopathies can occur for a wide array of underlying causes, symptoms vary significantly but may include:

• easy bleeding or bruising
• excessive bleeding after dental procedures, minor injuries, or surgery
• frequent nosebleeds
• heavy menstrual bleeding
• joint stiffness, tightness, warmth and pain

How Is Coagulopathy Diagnosed?

If coagulopathy is suspected, some simple laboratory tests can help narrow down a diagnosis. Depending on the results, further testing may be needed. Initial tests may include:

• complete blood count to analyze all blood components including platelets
• peripheral blood smear, in which a blood sample is examined under a microscope to look for signs of disease or evaluate blood cell abnormalities
• prothrombin time (PT) and partial thromboplastin time (PTT) blood clotting tests

Coagulopathy Treatments

Treatment for a blood clotting disorder depends on the type, severity and underlying cause. In addition to treating any disease, injury or other condition responsible for blood clotting problems, treatment may include:

• blood clotting factor infusions
• fibrinogen replacement therapy
• platelet transfusions