Written and peer-reviewed by physicians—but use at your own risk. Read our disclaimer.

banner image

amboss

Trusted medical answers—in seconds.

Get access to 1,000+ medical articles with instant search
and clinical tools.

Try free for 5 days

Disseminated intravascular coagulation

Last updated: January 20, 2021

Summary

Disseminated intravascular coagulation (DIC) is a disorder characterized by systemic activation of the clotting cascade with microthrombi formation, platelet consumption, and subsequent exhaustion of all clotting factors. Common causes of DIC include sepsis, trauma, and malignancy. Depending on the underlying pathophysiological processes and subtype, patients present with signs of bleeding (e.g., purpura, petechiae, ecchymoses) and/or multiorgan failure. Laboratory studies can be highly variable. However, typical findings in overt decompensated DIC include thrombocytopenia, prolonged PT and aPTT, decreased fibrinogen levels, and elevated D-dimer levels. Treatment involves identification of the underlying cause and supportive therapy with transfusions of blood products (pRBCs, platelet concentrates, FFP, and/or cryoprecipitate) and, in select cases, anticoagulants (e.g., heparin) or antifibrinolytic therapy (tranexamic acid). Close monitoring and specialist consultation (e.g., critical care and hematology) are typically required.

Overview

Definition [1]

  • Disseminated intravascular coagulation (DIC): a syndrome characterized by thrombosis, hemorrhage, and organ dysfunction caused by systemic activation of the clotting cascade, which leads to platelet consumption and exhaustion of clotting factors
    • Latent DIC: no overt symptoms (little to no bleeding, increased risk of thrombotic events, laboratory abnormalities)
    • Overt DIC: clear signs and symptoms (e.g., bleeding, thrombosis) that depend on the balance of the deranged processes
  • Consumption coagulopathy: hyperfibrinolysis, causing overt coagulopathy and bleeding, PLUS hypercoagulability, which causes consumption of platelets and clotting factors, resulting in even more blood loss.

Classification [2][3]

The 2014 International Society of Thrombosis and Haemostasis (ISTH) harmonized guidelines distinguish between DIC types based on clinical phenotype, characteristic laboratory abnormalities, and treatment targets.

Overview of DIC types [1][3][4][5]
Types

Description

Suggestive laboratory findings Potential therapeutic targets
Nonsymptomatic type DIC
  • Symptoms: little to none
  • Predominant mechanism: low-grade fibrinolysis and/or hypercoagulation
  • Typical underlying diseases: variable
Bleeding type DIC
Massive bleeding type DIC
  • Symptoms: massive hemorrhage
  • Predominant mechanism: hyperfibrinolysis and consumption of platelets, clotting factors, and fibrinogen
  • Typical underlying diseases: trauma, obstetric complications, major surgical complications
Organ failure type DIC

TAT: Thrombin antithrombin complex, PPIC: Plasmin α2-plasmin inhibitor complex, PAI-1: Plasminogen activator inhibitor-1, PT: Prothrombin time, FDPs: Fibrin degradation products, FFP: Fresh frozen plasma, pRBC: Packed red blood cells

Etiology

The mnemonic “STOP Making Trouble!” helps to recall the etiology of DIC: S - Sepsis/Snakebites, T - Trauma (acute traumatic coagulopathy), O - Obstetric complications, P - Pancreatitis, M - Malignancy, T - Transfusion.

References:[1][4][6][7][8]

Pathophysiology

The pathophysiology of DIC is variable and depends on the underlying processes (see “Hemostasis and bleeding disorders” and “Acquired thrombophilia” for details). [3][4]

DIC is an acquired coagulopathy that is most frequently seen in hospitalized individuals.

Clinical features

Clinical features are variable and depend on the predominant underlying mechanism. [6]

The clinical features of DIC may appear acutely (e.g., following trauma, sepsis) or subacutely (e.g., DIC following malignancy).

Diagnostics

General principles [5][9]

  • Suspect DIC based on clinical features and typical underlying diseases; especially in patients with clinical deterioration.
  • Laboratory studies vary depending on the subtype, underlying disorders, and stage of DIC.
  • Challenges
    • No single test can reliably rule out DIC.
    • Nonsymptomatic DIC may only become apparent after specific laboratory screening.
    • DIC may already be irreversibly decompensated by the time diagnosis is made using scoring systems.
    • Many conditions can manifest with similar laboratory abnormalities (see “Differential diagnoses of DIC by laboratory findings”).

Laboratory studies [3][5][10]

Routine laboratory tests

The diagnosis of DIC is not based on a single marker but on a combination of laboratory findings. Thrombocytopenia, elevated D-dimer, increased PT and aPTT, and low fibrinogen should immediately raise suspicion for DIC. [10]

Specialized tests [3]

The following tests should be considered on a case-by-case basis in consultation with a specialist (e.g., intensivist, hematologist).

International Society of Thrombosis and Haemostasis (ISTH) DIC score [3][10][12]

  • Multiple scoring systems exist, each with advantages and disadvantages that depend on the underlying cause of DIC. [8]
  • The ISTH DIC score identifies patients with a high likelihood of overt DIC. It can only be used in patients with a known predisposing disease.

ISTH DIC score [13]

Criteria Level Points assigned
Platelet count > 100,000/mm3 0
50,000–100,000/mm3 1
< 50,000/mm3 2
Increase in fibrin markers (D-dimer, soluble fibrin, other FDPs) None 0
Moderate 2
Strong 3
Prothrombin time prolongation < 3 seconds 0
3–6 seconds 1
> 6 seconds 2
Fibrinogen ≥ 100 mg/dL 0
< 100 mg/dL 1

Interpretation

Differential diagnoses

Differential diagnoses by laboratory findings [14]

Differential diagnoses of DIC by laboratory findings [5][14][15]

Condition Platelets Bleeding time PT aPTT Fibrinogen D-dimer/FDPs
DIC Typically low but can be variable [16]
Thrombotic microangiopathy (TTP, HUS, HELLP syndrome)

↓↓

Normal

Normal

Normal

Normal or ↑
HIT

Normal or ↑

Normal or ↑

Normal

ITP

Normal

Normal

Normal

Normal or ↑
Liver disease

Normal or ↓

Normal or ↑

Normal or ↓

Normal or ↑
Vitamin K deficiency or warfarin use

Normal

Normal

Normal or ↑

Normal

Normal

Differential diagnoses by underlying process

The differential diagnoses listed here are not exhaustive.

Treatment

General principles [3][10]

  • First-line: treatment of the underlying disease (see “Etiology”)
  • Additional treatment depends on the subtype and is complicated (see “Overview of DIC types”). Involve specialists early.
  • Supportive therapy
    • Avoid invasive procedures if possible
    • Vitamin K supplementation if deficiency is suspected [1]
  • Initiate clinical and serial laboratory monitoring to assess response to treatment.

Treatment recommendations by type of DIC [1][3]

Type Generally recommended Generally discouraged
Nonsymptomatic type DIC
Organ failure type DIC
  • Anticoagulation: Consider therapeutic heparin.
  • Consider advanced treatments.
Bleeding type DIC
Massive bleeding type DIC

Treatment of the underlying disease is the cornerstone of management.

Blood products [2][3][5][10][10]

The decision to transfuse blood products should primarily be based on the presence or risk of bleeding. Laboratory parameters can be used as a treatment goal but should not be considered the sole rationale for transfusion. Involve specialists early.

Antifibrinolytic therapy [2][3]

Aminocaproic acid is generally contraindicated in DIC.

Anticoagulation [2][3][10][12][21]

Patients receiving anticoagulants require careful monitoring of bleeding events and dosage adjustment for BMI and kidney function.

Warfarin is ineffective and possibly harmful in DIC, as it inhibits production of protein C and protein S, which can worsen coagulopathy. [23][24]

Fibrinolytic therapy (e.g., tPA) should be avoided in all types of DIC. [1][3]

Acute management checklist

All patients [2][3]

  • Consider DIC in patients with an associated underlying condition and clinical deterioration.
  • Order CBC and serial coagulation tests (including PT, aPTT, fibrinogen, D-dimer and other FDPs).
  • Consider calculating the ISTH DIC score to support the diagnosis.
  • Rule out differential diagnoses.
  • Consult hematology and intensive care as needed.
  • Consider ordering specialized tests according to DIC type.
  • Identify and treat the underlying cause.
  • Monitor coagulation parameters frequently.
  • Avoid invasive procedures if possible.
  • Supplement vitamin K if there is a suspected deficiency.

Bleeding type DIC or massive bleeding type DIC

Nonsymptomatic type DIC and organ failure type DIC

References

  1. Asakura H. Classifying types of disseminated intravascular coagulation: clinical and animal models. J Intensive Care. 2014; 2 (1): p.20. doi: 10.1186/2052-0492-2-20 . | Open in Read by QxMD
  2. Kasper DL, Fauci AS, Hauser SL, Longo DL, Lameson JL, Loscalzo J. Harrison's Principles of Internal Medicine. McGraw-Hill Education ; 2015
  3. Levi MM. Disseminated Intravascular Coagulation. In: Nagalla S, Disseminated Intravascular Coagulation. New York, NY: WebMD. http://emedicine.medscape.com/article/199627. Updated: September 29, 2016. Accessed: March 27, 2017.
  4. Asakura H, et al. Proposal for new diagnostic criteria for DIC from the Japanese Society on Thrombosis and Hemostasis. Thromb J. 2016; 14 (1). doi: 10.1186/s12959-016-0117-x . | Open in Read by QxMD
  5. Papageorgiou C, et al. Disseminated Intravascular Coagulation: An Update on Pathogenesis, Diagnosis, and Therapeutic Strategies. Clinical and Applied Thrombosis/Hemostasis. 2018; 24 (9_suppl): p.8S-28S. doi: 10.1177/1076029618806424 . | Open in Read by QxMD
  6. Wada H, Matsumoto T, Yamashita Y. Diagnosis and treatment of disseminated intravascular coagulation (DIC) according to four DIC guidelines. J Intensive Care. 2014; 2 (1): p.15. doi: 10.1186/2052-0492-2-15 . | Open in Read by QxMD
  7. Levi M, et al. Guidelines for the diagnosis and management of disseminated intravascular coagulation. British Committee for Standards in Haematology.. Br J Haematol. 2009; 145 (1): p.24-33. doi: 10.1111/j.1365-2141.2009.07600.x . | Open in Read by QxMD
  8. Wada H, et al. Guidance for diagnosis and treatment of disseminated intravascular coagulation from harmonization of the recommendations from three guidelines. Journal of Thrombosis and Haemostasis. 2013; 11 (4): p.761-767. doi: 10.1111/jth.12155 . | Open in Read by QxMD
  9. Thachil J. Disseminated Intravascular Coagulation. Anesthesiology. 2016; 125 (1): p.230-236. doi: 10.1097/aln.0000000000001123 . | Open in Read by QxMD
  10. Salyer SW. Hematologic Emergencies. Elsevier ; 2007 : p. 555-574
  11. Levi M, Scully M. How I treat disseminated intravascular coagulation. Blood. 2018; 131 (8): p.845-854. doi: 10.1182/blood-2017-10-804096 . | Open in Read by QxMD
  12. Szczepiorkowski ZM, Dunbar NM. Transfusion guidelines: when to transfuse. Hematology Am Soc Hematol Educ Program. 2013; 2013 (1): p.638-644. doi: 10.1182/asheducation-2013.1.638 . | Open in Read by QxMD
  13. Nascimento B, Goodnough LT, Levy JH. Cryoprecipitate therapy. Br J Anaesth. 2014; 113 (6): p.922-934. doi: 10.1093/bja/aeu158 . | Open in Read by QxMD
  14. Gando S, Levi M, Toh C-H. Disseminated intravascular coagulation. Nat Rev Dis Primers. 2016; 2 (1). doi: 10.1038/nrdp.2016.37 . | Open in Read by QxMD
  15. Di Nisio M, et al. Diagnosis and treatment of disseminated intravascular coagulation: Guidelines of the Italian Society for Haemostasis and Thrombosis (SISET). Thromb Res. 2012; 129 (5): p.e177-e184. doi: 10.1016/j.thromres.2011.08.028 . | Open in Read by QxMD
  16. Wada H, et al. Expert consensus for the treatment of disseminated intravascular coagulation in Japan. Thromb Res. 2010; 125 (1). doi: 10.1016/j.thromres.2009.08.017 . | Open in Read by QxMD
  17. Kahn SR, Lim W, Dunn AS, et al. Prevention of VTE in Nonsurgical Patients. Chest. 2012; 141 (2): p.e195S-e226S. doi: 10.1378/chest.11-2296 . | Open in Read by QxMD
  18. Koba S, Yamaguchi T, Miki K, Makihara H, Imashuku S. Management of Chronic Disseminated Intravascular Coagulation Associated with Aortic Aneurysm/Dissection. Case Rep Hematol. 2019; 2019 : p.1-6. doi: 10.1155/2019/6204652 . | Open in Read by QxMD
  19. Rubin RN, Colman RW. Disseminated Intravascular Coagulation. Drugs. 1992; 44 (6): p.963-971. doi: 10.2165/00003495-199244060-00005 . | Open in Read by QxMD
  20. Müller MC, Meijers JC, Vroom MB, et al. Utility of thromboelastography and/or thromboelastometry in adults with sepsis: a systematic review. Crit Care. 2014; 18 (1): p.R30. doi: 10.1186/cc13721 . | Open in Read by QxMD
  21. Müller MCA, Meijers JC, van Meenen DM, Thachil J, et al. Thromboelastometry in critically ill patients with disseminated intravascular coagulation.. Blood Coagul Fibrinolysis. 2019; 30 (5): p.181-187. doi: 10.1097/MBC.0000000000000808 . | Open in Read by QxMD
  22. Whiting P, Al M, Westwood M, et al. Viscoelastic point-of-care testing to assist with the diagnosis, management and monitoring of haemostasis: a systematic review and cost-effectiveness analysis. Health Technol Assess (Rockv). 2015; 19 (58): p.1-228. doi: 10.3310/hta19580 . | Open in Read by QxMD
  23. Wikkelsø A, Wetterslev J, Møller AM, Afshari A. Thromboelastography (TEG) or rotational thromboelastometry (ROTEM) to monitor haemostatic treatment in bleeding patients: a systematic review with meta-analysis and trial sequential analysis. Anaesthesia. 2017; 72 (4): p.519-531. doi: 10.1111/anae.13765 . | Open in Read by QxMD
  24. Fahrendorff M, Oliveri RS, Johansson PI. The use of viscoelastic haemostatic assays in goal-directing treatment with allogeneic blood products – A systematic review and meta-analysis. Scandinavian Journal of Trauma, Resuscitation and Emergency Medicine. 2017; 25 (1). doi: 10.1186/s13049-017-0378-9 . | Open in Read by QxMD
  25. Whiting D, DiNardo JA. TEG and ROTEM: Technology and clinical applications. Am J Hematol. 2014; 89 (2): p.228-232. doi: 10.1002/ajh.23599 . | Open in Read by QxMD
  26. Taylor FB Jr, et al. Towards definition, clinical and laboratory criteria, and a scoring system for disseminated intravascular coagulation.. Thromb Haemost. 2001; 86 (5): p.1327-30. doi: 10.1055/s-0037-1616068 . | Open in Read by QxMD
  27. Boral BM, Williams DJ, Boral LI. Disseminated Intravascular Coagulation. Am J Clin Pathol. 2016; 146 (6): p.670-680. doi: 10.1093/ajcp/aqw195 . | Open in Read by QxMD
  28. Hunt BJ. Bleeding and Coagulopathies in Critical Care. N Engl J Med. 2014; 370 (9): p.847-859. doi: 10.1056/nejmra1208626 . | Open in Read by QxMD
  29. Levi M, Sivapalaratnam S. Disseminated intravascular coagulation: an update on pathogenesis and diagnosis. Expert Rev. Hematol. 2018; 11 (8): p.663-672. doi: 10.1080/17474086.2018.1500173 . | Open in Read by QxMD
  30. Wada H, et al. Differences and similarities between disseminated intravascular coagulation and thrombotic microangiopathy. Thromb J. 2018; 16 (1). doi: 10.1186/s12959-018-0168-2 . | Open in Read by QxMD
  31. Shah PB. Intention-to-treat and per-protocol analysis. Can Med Assoc J. 2011; 183 (6): p.696-696. doi: 10.1503/cmaj.111-2033 . | Open in Read by QxMD