Disseminated Intravascular Coagulation Clinical Presentation

The symptoms of disseminated intravascular coagulation (DIC) are often those of the underlying inciting condition (see Causes). In addition, symptoms of thrombosis, embolism, organ dysfunction, or bleeding may be present.

• Sepsis/severe infection (any microorganism)
• Trauma (eg, polytrauma, neurotrauma, fat embolism)
• Organ destruction (eg, severe pancreatitis)
• Malignancy

  • Solid tumors
  • Myeloproliferative/lymphoproliferative malignancies
• Obstetric calamities

  • Amniotic fluid embolism
  • Abruptio placentae
• Vascular abnormalities

  • Kasabach-Merritt syndrome
  • Large vascular aneurysms
• Severe hepatic failure
• Severe toxic or immunologic reactions

  • Snake bites
  • Recreational drugs
  • Transfusion reactions
  • Transplant rejection

Acute disseminated intravascular coagulation (DIC): The physical findings associated with disseminated intravascular coagulation (DIC) are usually those of the underlying or inciting etiology; however, patients with the acute disease (ie, hemorrhagic variety associated with excess plasmin formation) have petechiae on the soft palate, trunk, and extremities from thrombocytopenia and ecchymosis at venipuncture sites. These patients also manifest with ecchymosis in traumatized areas.

Chronic or subacute disseminated intravascular coagulation (DIC): In patients with so-called chronic or subacute disseminated intravascular coagulation (DIC) whose manifestation is thrombosis from excess thrombin formation, the symptoms and signs of venous thromboembolism may be present.

Several disease states may lead to the development of disseminated intravascular coagulation (DIC). In general, 2 major pathways may cause disseminated intravascular coagulation (DIC): (1) a systemic inflammatory response, leading to activation of the cytokine network and subsequent activation of coagulation (eg, in sepsis or major trauma); and/or (2) release or exposure of procoagulant material into the bloodstream (eg, in cancer or in obstetric cases). In some situations, both pathways may be present (eg, major trauma or severe necrotizing pancreatitis). Some of the most frequently occurring conditions are outlined below.

• Bacterial infection, in particular septicemia, is commonly associated with disseminated intravascular coagulation (DIC). No difference exists in the incidence of disseminated intravascular coagulation (DIC) in patients with gram-negative sepsis or gram-positive sepsis. In addition, systemic infections with other microorganisms, such as viruses and parasites, may lead to disseminated intravascular coagulation (DIC) as well.
• Factors involved in the development of disseminated intravascular coagulation (DIC) in patients with infections may be specific cell membrane components of the microorganism (lipopolysaccharide or endotoxin) or bacterial exotoxins (eg, staphylococcal alpha toxin). These components cause a generalized inflammatory response, characterized by the systemic occurrence of proinflammatory cytokines.
• Severe trauma is another clinical condition frequently associated with disseminated intravascular coagulation (DIC). A combination of mechanisms—including release of tissue material (fat, phospholipids) into the circulation, hemolysis, and endothelial damage—may contribute to the systemic activation of coagulation. In addition, solid evidence indicates that cytokines play a pivotal role in the occurrence of disseminated intravascular coagulation (DIC) in trauma patients as well. In fact, systemic cytokine patterns have been shown to be virtually identical in trauma patients and septic patients.
• Both solid tumors and hematologic malignancies may be complicated by disseminated intravascular coagulation (DIC). The mechanism of the derangement of the coagulation system in this situation is poorly understood. Solid tumor cells can express different procoagulant molecules, including tissue factor and a cancer procoagulant, a cysteine protease with factor X–activating properties. Cancer procoagulant is found in extracts of neoplastic cells and in the plasma of patients with solid tumors.
• Some tumors are associated with a form of disseminated intravascular coagulation (DIC) that is characterized by severe hyperfibrinolysis on top of an activated coagulation system. For example, this is the case in acute promyelocytic leukemia and some forms of prostatic cancer. Although clinically bleeding predominates in this situation, disseminated thrombosis is found in a considerable number of patients at autopsy.
• Acute disseminated intravascular coagulation (DIC) occurs in obstetric calamities such as placental abruption and amniotic fluid emboli. Amniotic fluid has been shown to be able to activate coagulation in vitro, and the degree of placental separation correlates with the extent of the disseminated intravascular coagulation (DIC), suggesting that leakage of thromboplastinlike material from the placental system is responsible for the occurrence of disseminated intravascular coagulation (DIC).
• Although the coagulation system may be activated in patients with preeclampsia, and HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome, clinically significant disseminated intravascular coagulation (DIC) only occurs in a small percentage of patients, usually with secondary complications.
• Vascular disorders, such as large aortic aneurysms or giant hemangiomas (Kasabach-Merritt syndrome), may result in local activation of coagulation. Activated coagulation factors can ultimately "overflow" to the systemic circulation and cause disseminated intravascular coagulation (DIC) but more common is the systemic depletion of coagulation factors and platelets as a result of local consumption.
• Other causes of disseminated intravascular coagulation (DIC) include severe toxic or immunologic reactions (eg, transfusion reactions) or severe inflammation (eg, acute pancreatitis).