Coagulopathy

Coagulopathy is a condition where your body has problems making blood clots properly. This can lead to excessive bleeding or, in some cases, too much clotting. While some people inherit these conditions from their parents, others develop them later in life due to illness, injury, or medication.

Table of contents

• What is coagulopathy?
• Types of coagulopathy
• Causes and risk factors
• Signs and symptoms
• Diagnosis
• Treatment and management
• Living with coagulopathy
• Complications

What is coagulopathy?

Coagulopathy happens when there’s a problem with how your blood forms clots. Your blood normally forms clots to stop bleeding when you’re injured. This clot-making process is called hemostasis^[11].

There are two main processes involved in forming blood clots. First, small cell fragments called platelets rush to the site of injury and form a temporary plug. Second, proteins in your blood work together to form a strong mesh that covers the platelet plug. These proteins are called clotting factors or coagulation factors. You need both platelets and the protein mesh for a strong clot^[11].

While platelet disorders are a dysfunction of the cells that stop bleeding, other coagulopathies result from inadequate or dysfunctional proteins. This is also called a blood clotting disorder^[11].

Blood clotting disorder, hypercoagulable state, thrombophilia, bleeding disorder

Types of coagulopathy

Coagulation disorders are problems in the body’s ability to control how the blood clots. If you have a clotting disorder, your blood may not clot enough, which can lead to too much bleeding, or your blood may form clots even without an injury^[2].

Bleeding disorders are conditions where the body is unable to form blood clots properly. These conditions are typically due to issues with blood clotting factors. This can result in excessive bleeding from not forming enough clots^[17].

Hypercoagulability describes excessive blood clotting, which can disrupt blood flow and increase the risk of problems that include deep vein thrombosis or pulmonary embolism^[17].

People can either inherit or acquire coagulation disorders. This means individuals can get the condition from their parents or develop one during their lifetime, often from another condition or a medicine affecting blood clotting factors^[17].

Common types of coagulopathy

Hemophilia is a genetic disorder that passes through families and prevents proper blood clotting. It causes excessive bleeding, swelling, and bruising. The most common types are hemophilia A and B, where the body lacks certain proteins in the blood for clotting^[17].

Von Willebrand disease is another genetic disorder that prevents clotting due to insufficient von Willebrand factor, a blood-clotting protein. Females are more likely to notice the condition than males due to heavy bleeding during menstrual periods or birth^[17].

Factor V Leiden and prothrombin gene mutation (G20210A) are the most commonly identified genetic defects that increase your risk for blood clotting. About 3% to 8% of people with ancestors from Europe have a copy of the factor V Leiden mutation^[1].

Antiphospholipid syndrome (APS) is an acquired condition that increases your blood clot risk^[2].

Disseminated intravascular coagulation (DIC) is when overactive proteins in the blood cause unnecessary clotting that can block blood flow. This can cause a stroke^[3].

Liver disease-associated bleeding can occur when liver disease causes scarring that increases the risk of bleeding disorders or thrombosis^[17].

Vitamin K deficiency bleeding occurs because the body requires vitamin K to form blood clots. Babies born with low vitamin K levels who do not receive supplements may develop vitamin K deficiencies^[17].

Causes and risk factors

Coagulopathy happens when there’s a problem in the blood clotting process. Usually, this happens because you don’t have enough of a certain protein (clotting factor) to make a stable clot^[11].

Hypercoagulable states are usually genetic (inherited from parents) or acquired conditions. The genetic form of this disorder means you’re born with the tendency to form blood clots. Acquired conditions are usually a result of surgery, trauma, medications or a medical condition that increases the risk of forming clots^[1].

Inherited conditions

Inherited hypercoagulable conditions include Factor V Leiden (the most common), prothrombin gene mutation (G20210A), deficiencies of natural proteins that prevent clotting (such as antithrombin, protein C and protein S), elevated levels of fibrinogen or dysfunctional fibrinogen, elevated levels of factor VIII and other factors including factor IX and XI, and abnormal fibrinolytic system^[1].

Acquired conditions

Acquired blood clotting disorders include antiphospholipid syndrome (APS) and disseminated intravascular coagulation^[1]. There are a range of conditions (including bleeding disorders), events and even medicines that can keep your body from making stable clots. They include cancer, especially some forms of leukemia, medicines like heparin, warfarin, other anticoagulants and even aspirin, platelet disorders (can be inherited from a family member or develop later as an autoimmune disease), rare factor deficiencies, severe liver disease, von Willebrand disease, and vitamin K deficiency^[11].

Risk factors

Some coagulopathies are inherited. This means that you’re at increased risk if you have a history of bleeding disorders in your biological family. Taking blood thinners, like heparin or warfarin, may also increase your risk. These medicines prevent dangerous clots. But they may lead to excess bleeding if you’re injured^[11].

The following factors increase your risk of developing a blood clot: obesity, pregnancy, immobility (including prolonged inactivity, long trips by plane or car), smoking, oral contraceptives, certain cancers, trauma, certain surgeries, age (increased risk for people over age 60), a family history of blood clots, chronic inflammatory diseases, diabetes, high blood pressure, high cholesterol, prior central line placement^[4].

Signs and symptoms

Coagulopathy disorders can cause prolonged bleeding from cuts or wounds on your skin. But they can involve internal bleeding, too. This can look and feel different from managing a scrape or a cut^[11].

Blood clotting disorder symptoms can vary depending on where in your body you have a blood clot^[1].

Bleeding disorder symptoms

Signs to watch out for include frequent nosebleeds or bleeding from your gums despite good oral hygiene, extensive bruising (a sign of blood beneath your skin), heavy period bleeding (periods that last more than seven days, result in saturating a pad/tampon more often than every two hours, passing clots larger than 1 inch wide), joints that are swollen, warm to the touch, stiff or harder to move, spitting, vomiting or coughing up blood (may look red, pink and frothy or like coffee grounds), black, tarry poop, headaches, vision loss or confusion (may be a sign of brain bleed)^[11].

Excessive clotting symptoms

Symptoms may include swelling, tenderness and pain in your leg can mean you have a deep vein thrombosis, chest pain with shortness of breath can mean a possible pulmonary embolism, heart attack, and stroke^[1].

Diagnosis

If you think you may have a blood clotting disorder, your doctor will ask about your family and medical history. They may also run tests to be sure of the diagnosis^[2].

The diagnostic is based on an objective analysis of the habits of a patient, for example the type of medication they’re taking, if they suffer from allergies, if they’ve had a blood transfusion and if they smoke or drink. It is also based on the medical history of the patient, blood analysis and tests to evaluate blood clotting^[7].

After starting treatment, your provider will check to make sure it is working. This may include blood tests right after treatment, and then regularly to see whether there is a change in the levels of clotting factors or antibodies within your blood^[16].

Treatment and management

If you have a blood clotting disorder, you may need medicine to stop the blood from clotting^[2]. Treatment includes fresh frozen plasma and clotting factor concentrates to replace the missing proteins^[11].

The haematologist can consider it to be necessary to prescribe medication such as medications that prevent the formation of blood clots, contraceptive pills to counteract the excessive bleeding during menstruation, or medication that substitutes protein in the blood to ensure you do not excessively bleed^[7].

Novel approaches, such as point-of-care testing (POC) and administration of coagulation factor concentrates (CFCs), aim to tailor the haemostatic therapy to each patient and thus reduce the risks of over- or under-transfusion. CFCs are an effective alternative to ratio-based transfusion therapies for the correction of different types of coagulopathies^[5].

In case of major bleeding or urgent surgery in patients treated with vitamin K antagonist anticoagulants, prothrombin complex concentrate (PCC) can effectively reverse the effects of the anticoagulant drug^[5].

In trauma-induced coagulopathy, fibrinogen concentrate is the preferred first-line treatment for hypofibrinogenaemia. Goal-directed coagulation management algorithms based on POC results provide guidance on how to adjust the treatment to the needs of the patient^[5].

Living with coagulopathy

After a blood clot, it’s important to follow your doctor’s advice and treatment plan. Generally, lifestyle tips will not only keep you healthy, but can help prevent future blood clots^[12].

Stay active

One of the risk factors for DVT is inactivity, including sitting for extended periods of time, such as on a long-haul flight or being immobile after a serious injury. Physical activity is crucial for maintaining muscle strength and joint health, which are essential for individuals with bleeding disorders. Regular exercise can help reduce the risk of joint bleeds and improve overall well-being^[13].

Medication adherence

Adhering to your medication regimen is vital for managing bleeding disorders effectively. Missing doses can lead to increased risk of bleeding episodes and other complications. To stay on track, consider using medication reminders such as using a bleeding disorder app or placing your due dates on your calendar^[13].

Diet and nutrition

Diet plays a crucial role in managing bleeding disorders. Lean meats, leafy greens, and whole grains should be staples in your diet. It’s equally important to avoid foods that might interfere with blood clotting, such as those high in vitamin E or omega-3 fatty acids^[13].

Hydration

Staying hydrated is essential for overall health, but it’s particularly important for individuals with bleeding disorders. Dehydration can lead to many negative outcomes, and this in turn could impact your bleeding disorder. Aim to drink plenty of water throughout the day^[13].

Routine follow-up care

How often you see your healthcare provider should depend on your bleeding disorder, your symptoms, and your treatment plan. Even if you do not have symptoms and are not undergoing a treatment, you should see your provider for ongoing care. Follow your treatment plan as directed by your provider to prevent complications^[16].

Complications

Yes, blood clotting disorders can be dangerous, especially when you don’t get treatment. People with coagulation disorders have an increased risk of getting a blood clot in their arteries (blood vessels that carry blood away from your heart) or veins (blood vessels that carry blood to your heart)^[1].

Blood clots in your veins can travel through your bloodstream and cause deep vein thrombosis (a blood clot in the veins of your pelvis, leg, arm, liver, intestines or kidneys) or a pulmonary embolus (blood clot in your lungs)^[1].

Blood clots in your arteries can increase your risk for stroke, heart attack, severe leg pain, difficulty walking, and loss of an arm or leg^[1].

Losing too much blood is life-threatening without prompt treatment. This is why you should go to the emergency room if you have signs of severe blood loss. Other types of bleeding, like bleeding into joints, aren’t life-threatening. But they can cause long-term problems, like permanently painful and limited joint movement^[11].

Yes, it’s possible to have a miscarriage if you have a blood clot disorder like antiphospholipid syndrome. This disorder increases your blood clot risk, especially if you’ve had blood clots before. Higher blood volume and pressure during pregnancy play a role in making you five times more likely to develop a blood clot, even if you don’t have a blood clotting disorder^[1].