Clinical Background
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/year in U.S.
Risk Factors
- Sepsis (bacterial, viral, fungal)
- Trauma (polytrauma, fat embolism, burns)
- Malignancy (solid tumors, myeloproliferative malignancies)
- Obstetric complications (abruptio placentae, placenta previa, amniotic fluid embolus)
- Toxic reactions (snake bites)
- Immunologic reactions (hemolytic transfusion reaction, transplant rejection)
- Organ destruction (pancreatitis, hepatic failure)
Pathophysiology
- Activation of the inflammatory pathway via cytokines
- Suppression of physiologic anticoagulant pathways
- Systemic generation of thrombin
- Impaired fibrinolysis
Clinical Presentation
- Hemorrhage - petechiae, purpura, epistaxis, mucous membrane bleeding
- Thrombosis
- May lead to organ failure
- Chronic DIC occurs in cancer patients
- Primary symptom is thrombosis
- Referred to as Trousseau syndrome
Treatment
- Treat underlying disorder causing DIC
- Replacement therapy until disorder resolved
- Platelets
- Fresh frozen plasma
- Patients with chronic DIC and thrombosis may require heparin therapy
See Also
Diagnosis
Diagnosis
- Patient has risk factors for DIC
- CBC - may demonstrate decreased platelets
- Prolonged clotting times
- PT - increased (may be normal in chronic DIC)
- PTT - increased (may be normal in chronic DIC)
- Thrombin time - increased
- Increased levels of fibrin-related markers
- D-dimer - increased in acute and chronic DIC
- Decreased - coagulation elements
- Platelet count - decreased
- Fibrinogen - decreased, but an acute phase reactant so it may not decrease until DIC is severe
| Coagulation Test Scoring Scoring system for DIC proposed by ISTH, International Society on Thrombosis and Hemostasis |
| Score | 0 | 1 | 2 | 3 |
| Platelet count (x10^9/L) | >100 | <100 | <50 | |
| PT (seconds) | <3 | >3 but <6 | greater than or equal to 6 | |
| Fibrinogen (g/L) | >1 | <1 | | |
| Fibrin-related markers* (increase) | No increase | | Moderate increase | Strong increase |
| TOTAL | If greater than or equal to 5, compatible with overt DIC - repeat scoring daily. If <5, suggestive of non-overt DIC - repeat scoring after 1-2 days. |
| *Cutoffs for scoring fibrin-related markers must be established for the specific assay. |
|
Risk Assessment
- Does the patient have an underlying disorder known to be associated with DIC?
- If no - stop
- If yes - check coagulation tests
- Order coagulation tests
- Platelet count, prothrombin time (PT), D-dimer and fibrinogen level
- Score global coagulation test results
- D-dimer measurements alone have excellent negative predictive value for ruling out DIC
Differential Diagnosis
- Idiopathic thrombocytopenic purpura
- Hemolytic uremic syndrome
- Thrombotic thrombocytopenic purpura
- Severe liver disease
- Malignancy
Tests generally appear in the order most useful for common clinical situations
| Test name: CBC with Platelet Count
|
| ARUP #: 0040002 |
| Methodology: Automated Cell Count
|
| Use: Screen for hemostasis |
| Limitations: |
| Follow-up:
|
| Test name: Prothrombin Time
|
| ARUP #: 0030215 |
| Methodology: Electromagnetic Mechanical Clot Detection
|
| Use: Screen for hemostasis |
| Limitations: |
| Follow-up:
|
| Test name: Partial Thromboplastin Time
|
| ARUP #: 0030235 |
| Methodology: Electromagnetic Mechanical Clot Detection
|
| Use: Screen for hemostasis |
| Limitations: |
| Follow-up:
|
| Test name: Thrombin Time with Reflex to Thrombin Time 1:1 Mix
|
| ARUP #: 0030260 |
| Methodology: Clotting
|
| Use: Screen for hemostasis |
| Limitations: |
| Follow-up:
|
| Test name: Fibrinogen
|
| ARUP #: 0030130 |
| Methodology: Electromagnetic Mechanical Clot Detection
|
| Use: Use with fibrinogen (activity) assay to diagnose dysfibrinogenemia |
| Limitations: |
| Follow-up:
|
| Test name: D-Dimer
|
| ARUP #: 0030057 |
| Methodology: Immunoturbidimetric
|
| Use: Diagnose DIC |
| Limitations: Presence of rheumatoid factor may lead to false-positives |
| Follow-up:
|
| Test name: Soluble Fibrin Monomer
|
| ARUP #: 0030126 |
| Methodology: Hemagglutination
|
| Use: Not recommended; order D-Dimer test instead |
| Limitations: |
| Follow-up:
|
Additional Tests Available
| Test name: Prothrombin Time with Reflex to PT 1:1 Mix
|
| ARUP #: 0030163 |
| Methodology: Electromagnetic Mechanical Clot Detection
|
| Comments: |
| Test name: Partial Thromboplastin Time with Reflex to PTT 1:1 Mix
|
| ARUP #: 0030162 |
| Methodology: Electromagnetic Mechanical Clot Detection
|
| Comments: |
| Test name: Partial Thromboplastin Time with Reflex to PTT 1:1 Mix & Platelet Neutralization Procedure
|
| ARUP #: 0030004 |
| Methodology: Clotting
|
| Comments: |
| Test name: Fibrinogen Panel
|
| ARUP #: 0030137 |
| Methodology: Refer to individual components
|
| Comments: |
| Test name: Platelet Antigen Genotyping Panel
|
| ARUP #: 0051308 |
| Methodology: Polymerase Chain Reaction/Fluorescence Monitoring
|
| Comments: |
References
Cited References
Franchini M, Lippi G, Manzato F. Recent acquisitions in the pathophysiology, diagnosis and treatment of disseminated intravascular coagulation. Thromb J.
2006;
4:
4-.
General References
Cauchie P, Cauchie Ch, Boudjeltia KZ, Carlier E, Deschepper N, Govaerts D, Migaud-Fressart M, Woodhams B, Brohee D. Diagnosis and prognosis of overt disseminated intravascular coagulation in a general hospital -- meaning of the ISTH score system, fibrin monomers, and lipoprotein-C-reactive protein complex formation. Am J Hematol.
2006;
81(
6):
414-419.
Dempfle CE. Disseminated intravascular coagulation and coagulation disorders. Curr Opin Anaesthesiol.
2004;
17(
2):
125-129.
Franchini M, Manzato F. Update on the treatment of disseminated intravascular coagulation. Hematology.
2004;
9(
2):
81-85.
Kruger D. Acute systemic disseminated intravascular coagulation: managing a complex medical condition. JAAPA.
2006;
19(
5):
28-32.
Labelle CA, Kitchens CS. Disseminated intravascular coagulation: treat the cause, not the lab values. Cleve Clin J Med.
2005;
72(
5):
377-5, 390.
Levi M, Opal SM. Coagulation abnormalities in critically ill patients. Crit Care.
2006;
10(
4):
222-.
Levi M. Disseminated intravascular coagulation. Crit Care Med.
2007;
35(
9):
2191-2195.
Levi M. Disseminated intravascular coagulation: What's new?. Crit Care Clin.
2005;
21(
3):
449-467.
Toh CH, Downey C. Back to the future: testing in disseminated intravascular coagulation. Blood Coagul Fibrinolysis.
2005;
16(
8):
535-542.
References from the ARUP Institute for Clinical and Experimental Pathology Research®
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.
Medical Reviewers
Lehman, Christopher M., M.D. Co-Medical Director, University Hospital Clinical Laboratory; Associate Professor, Clinical Pathology, University of Utah
Rodgers, George M. III, M.D., Ph.D. Medical Director, Hemostasis/Thrombosis at ARUP Laboratories; Professor, Medicine, Adjunct Professor, Pathology, University of Utah
Comprehensive Review: January 2008
Last Update: January 2008
