Description

• Acquired syndrome characterized by diffuse activation of intravascular coagulation. It can originate from and cause damage to the microvasculature, which, if sufficiently severe, can produce organ dysfunction.
• Occurs as a complication of pregnancy (e.g., abruptio placentae, fetus retention, amniotic fluid embolism), infection (especially gram-negative), malignancy (uncontrolled, metastatic tumor or leukemia), trauma, and other severe illnesses
• System(s) affected: Hematologic/Lymphatic/Immunologic
• Synonym(s): Consumptive coagulopathy; DIC

Epidemiology

Incidence
Unknown

• Predominant age: None
• Predominant sex: Male = Female

Risk Factors

See “Etiology.”

General Prevention

Aggressive interventions aimed at early treatment of the underlying clinical conditions

Pathophysiology

• Systemic formation of fibrin is the result of the simultaneous coexistence of:
  • Increased thrombin generation via tissue factor-/factor VII-mediated pathway
  • Suppression of the physiologic anticoagulant pathways:
    • Antithrombin due to consumption, degradation, and impaired synthesis
    • Proteins C and S due to decreased levels of thrombomodulin
  • Impaired fibrinolysis (early on):
    • Sustained increase in plasminogen activator inhibitor, type 1
• Increased fibrinolysis (late during the process) leads to bleeding.

Etiology

Causes can be classified as acute or chronic, systemic or localized:

• Sepsis/Severe infection (any microorganism)
• Trauma (polytrauma, neurotrauma)
• Obstetric complications (amniotic fluid embolism, abruptio placentae)
• Solid tumors and leukemias (especially acute promyelocytic leukemia)
• Vascular disorders, such as Kasabach-Merritt syndrome, large vascular aneurysms, and thrombosis
• Organ destruction (severe pancreatitis, severe liver failure)
• Severe toxic or immunologic reactions:
  • Snake bite
  • Recreational drugs
  • Transfusion reactions
  • Transplant rejection
  • Thermal injury
• Infant and adult respiratory distress syndrome
• Neonatal purpura fulminans

Commonly Associated Conditions

Thromboembolic phenomena are associated with venous thrombosis, thrombotic vegetations on the aortic heart valve, arterial emboli, and neonatal purpura fulminans (homozygous protein C or protein S deficiency).

Pediatric Considerations
Neonatal purpura fulminans is associated with DIC and protein C or protein S deficiency (homozygous).