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Soft Tissue Sarcoma (Thrombophilia)

Thrombophilia is the tendency for abnormal blood clotting. In a person with thrombophilia, a blood clot could form inside an unbroken blood vessel. If left unchecked, it can lead to loss of blood supply to tissues - sometimes with fatal consequences.

Thrombophilia is treatable. Treatments can prevent recurrent thrombosis in the future. The Thrombophilia Program at Children’s includes:

  • Diagnosis
  • Prevention treatment of thromboembolic events
  • Use of anticoagulant therapy in other conditions such as cardiac catheterization, cardiopulmonary bypass, dialysis, extra corporeal membrane oxygenation, stents and others

Children inherit thrombophilia from one parent or both parents. A genetic mutation can also cause it.

How do You Know if Your Child has Thrombophilia?

If he/she has a history of thrombosis, chances of having one or more forms of thrombophilia vary from less than one to 40 percent depending on:

  • Age
  • Sex
  • Ethnic background
  • Whether or not thrombosis was related to surgery, trauma, estrogen administration or prolonged bedrest

Reasons for increasing incidence and severity include:

  1. The capacity to cure most previously life-threatening diseases which then permits the development of new diseases such as thromboembolic events.
  2. The necessity for venous and arterial catheters to treat critically ill children.
  3. The availability of sensitive radiographic tools that can diagnose thromboembolic events in unusual locations which were previously undetected.
  4. The capacity to identify children with congenital prothrombotic disorders.

Developmental Hemostasis

  1. The coagulation system 
  2. Coagulation proteins 
  3. Coagulation inhibitors
  4. The fibrinolytic system
  5. The platelets and the vessel wall

Venous Thromboembolic Disease

  1. Incidence 
  2. Congenital prothrombotic disorders 
    • Factor V Leiden
    • Prothrombin mutant 
    • Protein C deficiency 
    • Protein S deficiency 
    • Antithrombin  
    • Dysfibrinogenemia 
    • Defects within the fibrinolytic system 
    • Platelet defect
    • Central venous catheters 
    • Underlying medical conditions 
    • Clinical presentation

    Arterial Thromboembolic Disease

    1. Incidence 
    2. Congenital causes of arterial disease 
      • Familial homozygous hypercholesterolemia 
      • Hyperhomocystinuria
      • Acquired disorders 
        • Takayasu's arteritis 
        • Occlusion of giant coronary aneurysm secondary to Kawasaki disease 
        • Occlusion of arteries in transplanted organs  
        • Acquired heart disease 
        • Paroxysmal nocturnal hemoglobinuria 
        • Central venous catheters
        • Clinical Presentation

        Antithrombotic Agents

        1. Heparin 
        2. Low molecular weight heparin 
        3. Coumadin

        Antiplatelet Therapy

        1. Aspirin
        2. Dipyridamole

        Thrombolytic Therapy

        1. Tissue plasminogen activator