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Coagulation and Hemostasis Disorders (Pediatric)

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What are coagulation and hemostasis disorders?

Disorders of coagulation and hemostasis include conditions where either blood does not clot and bleeding occurs or when thrombosis ensues secondary to increased clot formation. Coagulation or clot formation is the process by which blood changes from a liquid and becomes thicker and gel-like. When clot formation works effectively and efficiently, it’s crucial to healing a wound, and when it's impeded, bleeding results.

When clot formation occurs where it should not, a coagulate may form that blocks a blood vessel. This phenomenon is referred to as thrombosis. The formation of a blood clot or thrombus inside a blood vessel may block blood flow through the circulatory system and result in ischemia to tissues and organs such as a heart attack or stroke.

What causes coagulation and hemostasis disorders?

Disorders of coagulation and hemostasis may be passed along at birth or develop later. Hemophilia, the most common condition that leads to bleeding, is an example of a congenital condition (present from birth) where an important factor needed to make a blood clot is reduced or missing.

The main causes of thrombosis are conditions that make the blood clot more easily, injury to nearby tissues, and conditions that prevent normal blood flow. If a thrombus is large enough to reduce blood flow, it can keep some tissues from receiving oxygen. For example, a larger thrombus causing a greater obstruction to blood flow may result in tissue death or infarction. A blood clot that travels from one place to another in the body is called an embolus.

What are the symptoms of coagulation and hemostasis disorders?

Symptoms associated with a failure of blood clotting may include:

  • Easy bruising
  • Uncontrolled bleeding
  • Anemia
  • Swelling and pain

Symptoms associated with a thrombus formation may include:

  • Pain
  • Swelling
  • Infarction secondary to loss of appropriate blood flow
  • Dependent upon location of thrombus or embolus: heart attack, stroke, and pulmonary embolus with shortness of breath

How are coagulation and hemostasis disorders diagnosed?

  • Family history
  • History of present symptoms
  • Physical examination
  • Complete blood count
  • Screening blood tests: thrombin time, activated partial thromboplastin time, INR, fibrinogen
  • Identification of specific coagulation factor levels, i.e., factors that inhibit blood coagulation or clotting, and factors that promote clotting and thrombus formation

What is the treatment for coagulation and hemostasis disorders?

The most important aspect of treatment is to accurately diagnosis the etiology of bleeding or thrombus formation. Based on the diagnosis, treatment may include:

  • Infusions of missing factors needed to promote clotting
  • Blood thinners, anti-coagulants, such as heparin, lovenox, and aspirin to inhibit thrombus formation