Coagulation System Disorders

The coagulation system is made up of blood cells and proteins and is responsible for creating blood clots, which are an important part of the body’s healing process. When this system does not work properly, it can cause blood clots to form at inappropriate times, blocking the flow of blood to vital parts of the body.

Coagulation system disorders can affect any part of the body including the brain, abdomen, arms and legs. The major forms of coagulation disorders include:

  • Vitamin K deficiency
  • Liver disease 
  • Disseminated intravascular coagulation, which is when overactive proteins in the blood cause unnecessary clotting that can block blood flow. This can cause a stroke.
  • Development of circulating anticoagulants, which prevent the blood from clotting and can cause hemophilia

Symptoms

Symptoms associated with disorders of the coagulation system will vary depending on the type of disorder the patient has. Abnormal bleeding or the development of blood clots are the most common symptoms of most coagulation system disorders.

If the patient is experiencing vitamin K deficiency, symptoms may include:

  • Bleeding
  • Bruising more easily

Patients with liver disease may experience the following symptoms:

  • Yellowing of the skin (jaundice)
  • Pain in the upper right abdomen
  • Abdominal swelling
  • Nausea
  • Vomiting
  • Feeling unwell
  • Confusion
  • Sleepiness

Patients experiencing disseminated intravascular coagulation may notice the following symptoms:

  • Bleeding
  • Developing blood clots
  • Bruising easily
  • A drop in blood pressure

Bleeding is the most common symptom associated with the development of circulating anticoagulants. However, in rare cases a blood clot can form as a result of this disorder and patients may notice the following around the affected area:

  • Redness
  • Swelling
  • Tissue damage due to limited or blocked blood flow

Patients who have experienced a stroke due to a coagulation system disorder may display “classic” stroke symptoms including:

  • Sudden numbness or weakness of the face, arm or leg, especially on one side of the body
  • Sudden confusion
  • Sudden trouble speaking
  • Sudden trouble seeing in one or both eyes
  • Sudden trouble walking
  • Sudden dizziness, loss of balance or coordination
  • Sudden, severe headache with no known cause

 

Causes and Risk Factors

Disorders of the coagulation system can either be acquired after birth or they can be hereditary and passed down from the parents to the child. The most common type of hereditary coagulation disorder is hemophilia. Patients with hemophilia can be diagnosed at any age and the age of diagnosis is often associated with how severe the condition is. The more severe the condition is the younger a patient is when they are diagnosed.

Vitamin K deficiency is most commonly diagnosed in infants; however, it can affect patients of any age.

Risk factors associated with developing a coagulation system disorder include:

  • Parents with a hereditary coagulation disorder
  • Certain conditions, such as liver disease
  • Having a blood transfusion
  • Certain types of cancer
  • Blood infections
  • Severe trauma
  • Pregnancy complications

Diagnosis

Diagnosis of coagulation system disorders usually begins with a physical exam and a review of the patient's medical history and symptoms. A positive diagnosis of the condition is based on the patient's symptoms as well as the findings of a blood test. Other diagnostic tools may be used to better understand the patient's condition.

A blood test is the only way to positively diagnose a coagulation system disorder. The blood sample will be examined under a microscope to determine the following information:

  • The complete blood count (CBC)
  • How long it takes the blood to clot (Partial thromboplastin time)
  • How long it takes the liquid (plasma) portion of the blood to clot (Prothrombin time)
  • Whether there are coagulation elements present (fibrinogen)

Other diagnostic tools may include magnetic resonance imaging (MRI) and computed tomography (CT) angiography, which can be used to observe the condition and the area affected by disorder.

Treatments

Treatment of coagulation system disorders may include plasma transfusions or medications to help the blood clot or prevent the blood from clotting.

If a patient has experienced excessive bleeding, a plasma transfusion may be used to reintroduce blood clotting elements into the patient's blood.

Patients with a vitamin K deficiency are often prescribed phytonadione, a vitamin that boosts the level of vitamin K in the patient's body and helps the blood clot.

Anticoagulation medications, such as heparin, may be prescribed for patients who have trouble with overactive blood clotting. These anticoagulation medications may help prevent a stroke from occurring.

For patients who have experienced a stroke, the Stroke Program at Cedars-Sinai provides a multidisciplinary treatment approach through a personalized treatment plan tailored to each patient. Patient care is generally broken down into three categories: stroke prevention, treatment immediately after a stroke, and post-stroke rehabilitation.