Disseminated intravascular coagulation (DIC) is a disorder characterized by massive systemic activation of coagulation with consumption of platelets and coagulation proteins.

Epidemiology

• Incidence – >18,000 cases annually in U.S.

Risk Factors

• Sepsis (bacterial, viral, fungal)
• Trauma (polytrauma, fat embolism, burns)
• Malignancy (solid tumors, acute leukemia)
• Obstetric complications (abruptio placentae, placenta previa, amniotic fluid embolus)
• Toxic reactions (eg, venomous snake bite)
• Immunologic reactions (hemolytic transfusion reaction, transplant rejection)
• Organ destruction (pancreatitis, hepatic failure)

Pathophysiology

• Activation of coagulation pathways
  • Generation of thrombin and formation of fibrin in circulating blood
  • Consumption of coagulation factors and platelets
• Activation of inflammatory pathways via cytokines
• Suppression of physiologic anticoagulant pathways
• Activation and/or impairment of fibrinolysis

Clinical Presentation

• Generally occurs in the setting of a risk factor listed above
• Hemorrhage – petechiae, purpura, epistaxis, mucous membrane bleeding
• Thrombosis – may lead to organ failure
• Chronic DIC – occurs in cancer patients
  • Thrombosis is primary symptom
  • Referred to as Trousseau syndrome

Treatment

• Treat underlying disorder causing DIC
• Replacement therapy until underlying disorder resolved
  • Platelets
  • Fresh frozen plasma/cryoprecipitate
• Patients with chronic DIC and thrombosis may require heparin therapy

Indications for Testing

• Patient with risk factors for DIC and bleeding and/or thrombocytopenia; underlying disorder with known DIC association

Criteria for Diagnosis

• Score global coagulation test results based on the following scoring system proposed by the International Society on Thrombosis and Hemostasis (ISTH)
  • Platelet count (x10⁹/L)
    • (>100 = 0, 50-100 = 1, <50 = 2)
  • PT prolongation (seconds)
    • (<3 = 0, >3 but <6 = 1, ≥6 = 2)
  • Fibrinogen (g/L)
    • (>1 = 0, <1 = 1)
  • Fibrin-related markers (increase)
    • No increase = 0; moderate increase = 2; strong increase = 3
    • Cutoffs for scoring fibrin-related markers must be established for the specific assay
  • TOTAL
    • If ≥5, compatible with overt DIC – repeat scoring daily
    • If <5, suggestive of non-overt DIC – repeat scoring after 1-2 days

Laboratory Testing

• CBC –  thrombocytopenia frequently present
• Clotting times
  • Prothrombin time (PT) – prolonged (may be normal in early or chronic DIC)
  • Partial thromboplastin time (PTT) – prolonged (may be normal in early or chronic DIC)
  • Thrombin time (TT) – may be increased due to consumption of fibrinogen
• Fibrin-related markers
  • D-dimer – increased in acute and chronic DIC (best single test)
    • D-dimer measurements alone have excellent negative predictive value for ruling out DIC
    • A normal d-dimer essentially rules out DIC
    • Elevated d-dimer levels are seen in a number of conditions in addition to DIC (eg, pregnancy, acute thrombosis)
• Coagulation factors
  • Fibrinogen – decreased
    • As an acute phase reactant, fibrinogen might not decrease until DIC is severe

Differential Diagnosis

• Idiopathic thrombocytopenic purpura
• Hemolytic uremic syndrome
• Thrombotic thrombocytopenic purpura
• Severe liver disease
• Malignancy
• Drug-induced thrombocytopenia
• Splenic sequestration
• Heparin-induced thrombocytopenia

Tests generally appear in the order most useful for common clinical situations

General

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• Dalainas I. Pathogenesis, diagnosis, and management of disseminated intravascular coagulation: a literature review. Eur Rev Med Pharmacol Sci. 2008; 12 (1) :19-31.PubMed
• Franchini M, Lippi G, Manzato F. Recent acquisitions in the pathophysiology, diagnosis and treatment of disseminated intravascular coagulation. Thromb J. 2006; 4 (4) :1-9.PubMed
• Franchini M, Manzato F. Update on the treatment of disseminated intravascular coagulation. Hematology. 2004; 9 (2) :81-85.PubMed
• Kruger D. Acute systemic disseminated intravascular coagulation: managing a complex medical condition. JAAPA. 2006; 19 (5) :28-32.PubMed
• Labelle CA, Kitchens CS. Disseminated intravascular coagulation: treat the cause, not the lab values. Cleve Clin J Med. 2005; 72 (5) :377-5, 390.PubMed
• Levi M. Disseminated intravascular coagulation. Crit Care Med. 2007; 35 (9) :2191-2195.PubMed
• Toh CH, Downey C. Back to the future: testing in disseminated intravascular coagulation. Blood Coagul Fibrinolysis. 2005; 16 (8) :535-542.PubMed

ARUP Institute for Clinical and Experimental Pathology®

• Lehman CM, Wilson LW, Rodgers GM. Analytic validation and clinical evaluation of the STA LIATEST immunoturbidimetric D-dimer assay for the diagnosis of disseminated intravascular coagulation. Am J Clin Pathol. 2004; 122 (2) :178-184.PubMed
• Ness P, Creer M, Rodgers GM, Naoum JJ, Renkens K, Voils SA, Alexander WA. Building an immune-mediated coagulopathy consensus: early recognition and evaluation to enhance post-surgical patient safety. Patient Saf Surg. 2009; 3 (1) :8-.PubMed

Fetal and Neonatal Alloimmune Thrombocytopenia
Fibrinolytic Disorders
Functional Platelet Disorders
HELLP Syndrome
Hemolytic Anemias
Henoch Schönlein Purpura
Heparin-Associated Antibody Syndrome
Hereditary Hemorrhagic Telangiectasia
Hypercoagulable States
Sepsis in Newborns
Thrombotic Microangiopathies
Venous Thromboembolism