- Acquired syndrome characterized by diffuse activation of intravascular coagulation arising from different causes. It can originate from and cause damage to the microvasculature, which, if sufficiently severe, can produce organ dysfunction.
- Occurring in complications of obstetrics (eg, 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
- Predominant age: None
- Predominant sex: Male = Female
Aggressive interventions aimed at early treatment of the underlying clinical conditions
- 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.
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).
Neonatal purpura fulminans is associated with DIC and protein C or protein S deficiency (homozygous).
Symptoms and signs are related to the underlying disease process and disseminated intravascular coagulation.
Symptoms of microvascular thrombosis (e.g., renal failure), as well as diffuse bleeding
- Bleeding manifestations:
- Microvascular thrombosis:
- Skin (skin infarction, digital gangrene)
- Gastrointestinal (mucosal ulcerations, bowel infarction)
- Renal (oliguria, anuria, uremia)
- Pulmonary (hypoxemia, acute respiratory distress syndrome)
- Neurologic (convulsions, delirium, coma, multifocal cortical infarction)
Diagnostic Tests & Interpretation
No single laboratory test is sensitive and specific enough to allow a definitive diagnosis of DIC.
- Increased partial thromboplastin time (PTT)
- Increased prothrombin test (PT)
- Decreased fibrinogen (serial levels)
- Increased fibrin degradation product (FDP)
- Positive D-dimer
- Decreased antithrombin III, decreased protein C
- Microangiopathic hemolytic anemia (schistocytes, increased lactate dehydrogenase levels, low hemoglobin)
- Decreased factor VIII (can help differentiate DIC vs liver failure—normal in liver failure)
Initial lab tests
Diagnostic algorithm for overt DIC (International Society on Thrombosis and Haemostasis)
- Assess if underlying disease is known to be associated with DIC:
- YES: Proceed with this algorithm.
- NO: Do not use this algorithm.
- Order global coagulation tests (PT, PTT, fibrinogen, soluble fibrin monomers, or FDPs)
- Score global coagulation test results:
- Platelet count (>100 = 0, <100 = 1, <50 = 2)
- Elevated fibrin-related markers (no increase = 0, moderate increase = 1, strong increase = 3)
- Prolonged PT (<3 seconds = 0, >3 but <6 seconds = 1, >6 seconds = 2)
- Fibrinogen level (>1 g/l = 0, <1 g/l = 1)
- Calculate score:
- If ≥5: Compatible with DIC, repeat scoring daily. If <5: Suggestive (not affirmative for nonovert DIC: Repeat in 1–2 days).
Follow-Up & Special Considerations
Frequent follow-up of initially abnormal laboratory tests to see effect of therapeutic interventions
- Fulminant liver failure or massive hepatic necrosis
- Vitamin K deficiency
- Thrombotic thrombocytopenic purpura
- Hemolytic–uremic syndrome
- Heparin-induced thrombocytopenia
- Primary fibrinolysis
- HELLP syndrome in pregnancy (hemolysis, elevated liver function, and low platelets)
Heterogeneity of the underlying disorders and the clinical presentations makes the therapeutic approach to DIC difficult:
- Appropriate health care: Inpatient and often intensive care unit (depending on underlying condition)
- Treat underlying condition (e.g., evacuation of uterus in abruptio placentae; broad-spectrum antibiotics for gram-negative sepsis)
- Supportive care with transfusions in patients who are bleeding, going for surgery, or at high risk of bleeding. Do not treat abnormal laboratory parameters:
- Fresh frozen plasma
- Platelet concentrates
- Cryoprecipitate or fibrinogen concentrates
- Anticoagulants remain very controversial. Deep venous thrombosis prophylaxis is recommended in patients who are not bleeding.
- Restoration of anticoagulant pathways:
- Recombinant human-activated protein C (benefit only in carefully selected patients at high risk of death from sepsis. The drug should only be administered in an Intensive Care Unit (ICU) when patient is monitored by trained personnel) (1)[A].
- Activated factor VII use remains controversial.
- Broad-spectrum antibiotics for sepsis
- Recombinant human-activated protein C in severe sepsis (1)
Surgical treatment or procedures should be considered, especially if they are treating the underlying condition (e.g., evacuation of uterus in abruptio placentae; some trauma or bleeding situations).
- Treat underlying disorder.
- Frequent monitoring of clinical and laboratory response
Usually dictated by severity of underlying condition. Some cases can be managed on a standard ward, but ICU care is typical.
Once clinical and laboratory criteria are significantly improved and underlying reason for DIC is under control
- Monitor closely until much improved
- Serial platelet count, coagulation tests, and fibrinogen levels to see effect of therapeutic interventions
- Related to the severity of cause
- Decreased antithrombin level is a poor prognostic factor in DIC.
- Acute renal failure
- Cardiac tamponade
- Intracerebral hematoma
- Various thrombotic complications, including myocardial infarction, stroke, gangrene, and loss of digits
1. Vincent JL, Bernard GR, Beale R, et al. Drotrecogin alfa (activated) treatment in severe sepsis from the global open-label trial ENHANCE: further evidence for survival and safety and implications for early treatment. Crit Care Med. 2005;33:2266–77.
Franchini M, Lippi G, Manzato F. Recent acquisitions in the pathophysiology, diagnosis and treatment of disseminated intravascular coagulation. Thromb J.2006;4:4.
Levi M. Disseminated intravascular coagulation. Crit Care Med. 2007;35:2191–5.
Levi M, Toh CH, Thachil J, Watson HG et al. Guidelines for the diagnosis and management of disseminated intravascular coagulation. British Committee for Standards in Haematology. Br J Haematol. 2009;145:24–33.
Taylor FB, Toh CH, Hoots WK, et al. Towards definition, clinical and laboratory criteria, and a scoring system for disseminated intravascular coagulation.Thromb Haemost. 2001;86:1327–30.
286.6 Defibrination syndrome
67406007 Disseminated intravascular coagulation (disorder)
Treat underlying condition(s); transfusions represent supportive measures.
Transfusions are not indicated in patients with abnormal laboratory parameters without clinical bleeding.