Official reprint from UpToDate®
www.uptodate.com ©2017 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Clinical features, diagnosis, and treatment of disseminated intravascular coagulation in adults

Lawrence LK Leung, MD
Section Editor
Pier Mannuccio Mannucci, MD
Deputy Editor
Jennifer S Tirnauer, MD


Disseminated intravascular coagulation (DIC, also called consumption coagulopathy and defibrination syndrome) is a systemic process with the potential for causing thrombosis and hemorrhage. It can present as an acute, life-threatening emergency or a chronic, subclinical process, depending on the degree and tempo of the process and the contribution of morbidities from the underlying cause. Identifying DIC and the underlying condition responsible for it are critical to proper management.

This topic discusses the pathogenesis, clinical manifestations, diagnosis, treatment, and prognosis of DIC in adults. DIC in children and during pregnancy are discussed in detail separately. (See "Disseminated intravascular coagulation in infants and children" and "Disseminated intravascular coagulation during pregnancy".)

Separate topic reviews also discuss general approaches to evaluating patients with abnormal bleeding and unexplained microangiopathic hemolytic anemia and thrombocytopenia. (See "Approach to the adult patient with a bleeding diathesis" and "Approach to the patient with suspected TTP, HUS, or other thrombotic microangiopathy (TMA)".)


Intravascular coagulation and fibrinolysis — Normal hemostasis ensures formation of a blood clot at the site of vessel injury, followed by resolution of the clot to allow tissue repair. There are multiple feedbacks built into this system to prevent activation of coagulation in the absence of vessel injury and restrict the clot to the site of injury. (See "Overview of hemostasis".)

In DIC, the processes of coagulation and fibrinolysis become abnormally (and often massively) activated within the vasculature, leading to ongoing coagulation and fibrinolysis.

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:

Subscribers log in here

Literature review current through: Nov 2017. | This topic last updated: Aug 29, 2017.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2017 UpToDate, Inc.
  1. Hellum M, Øvstebø R, Brusletto BS, et al. Microparticle-associated tissue factor activity correlates with plasma levels of bacterial lipopolysaccharides in meningococcal septic shock. Thromb Res 2014; 133:507.
  2. Nieuwland R, Berckmans RJ, McGregor S, et al. Cellular origin and procoagulant properties of microparticles in meningococcal sepsis. Blood 2000; 95:930.
  3. Gordon SG, Franks JJ, Lewis B. Cancer procoagulant A: a factor X activating procoagulant from malignant tissue. Thromb Res 1975; 6:127.
  4. Gordon SG, Mielicki WP. Cancer procoagulant: a factor X activator, tumor marker and growth factor from malignant tissue. Blood Coagul Fibrinolysis 1997; 8:73.
  5. Capon SM, Goldfinger D. Acute hemolytic transfusion reaction, a paradigm of the systemic inflammatory response: new insights into pathophysiology and treatment. Transfusion 1995; 35:513.
  6. Levi M, Toh CH, Thachil J, Watson HG. Guidelines for the diagnosis and management of disseminated intravascular coagulation. British Committee for Standards in Haematology. Br J Haematol 2009; 145:24.
  7. Liaw PC, Ito T, Iba T, et al. DAMP and DIC: The role of extracellular DNA and DNA-binding proteins in the pathogenesis of DIC. Blood Rev 2016; 30:257.
  8. Martinod K, Wagner DD. Thrombosis: tangled up in NETs. Blood 2014; 123:2768.
  9. Martinod K, Demers M, Fuchs TA, et al. Neutrophil histone modification by peptidylarginine deiminase 4 is critical for deep vein thrombosis in mice. Proc Natl Acad Sci U S A 2013; 110:8674.
  10. Xu J, Zhang X, Pelayo R, et al. Extracellular histones are major mediators of death in sepsis. Nat Med 2009; 15:1318.
  11. Kim JE, Lee N, Gu JY, et al. Circulating levels of DNA-histone complex and dsDNA are independent prognostic factors of disseminated intravascular coagulation. Thromb Res 2015; 135:1064.
  12. Mant MJ, King EG. Severe, acute disseminated intravascular coagulation. A reappraisal of its pathophysiology, clinical significance and therapy based on 47 patients. Am J Med 1979; 67:557.
  13. Sack GH Jr, Levin J, Bell WR. Trousseau's syndrome and other manifestations of chronic disseminated coagulopathy in patients with neoplasms: clinical, pathophysiologic, and therapeutic features. Medicine (Baltimore) 1977; 56:1.
  14. Ghosh K, Shetty S. Blood coagulation in falciparum malaria--a review. Parasitol Res 2008; 102:571.
  15. Siegal T, Seligsohn U, Aghai E, Modan M. Clinical and laboratory aspects of disseminated intravascular coagulation (DIC): a study of 118 cases. Thromb Haemost 1978; 39:122.
  16. Spero JA, Lewis JH, Hasiba U. Disseminated intravascular coagulation. Findings in 346 patients. Thromb Haemost 1980; 43:28.
  17. Taylor FB Jr, 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.
  18. Isbister GK. Snakebite doesn't cause disseminated intravascular coagulation: coagulopathy and thrombotic microangiopathy in snake envenoming. Semin Thromb Hemost 2010; 36:444.
  19. Matsuda T. Clinical aspects of DIC--disseminated intravascular coagulation. Pol J Pharmacol 1996; 48:73.
  20. Levi M, Ten Cate H. Disseminated intravascular coagulation. N Engl J Med 1999; 341:586.
  21. Sallah S, Wan JY, Nguyen NP, et al. Disseminated intravascular coagulation in solid tumors: clinical and pathologic study. Thromb Haemost 2001; 86:828.
  22. Smith OP, White B, Vaughan D, et al. Use of protein-C concentrate, heparin, and haemodiafiltration in meningococcus-induced purpura fulminans. Lancet 1997; 350:1590.
  23. Gando S, Nanzaki S, Kemmotsu O. Disseminated intravascular coagulation and sustained systemic inflammatory response syndrome predict organ dysfunctions after trauma: application of clinical decision analysis. Ann Surg 1999; 229:121.
  24. Hulka F, Mullins RJ, Frank EH. Blunt brain injury activates the coagulation process. Arch Surg 1996; 131:923.
  25. Selladurai BM, Vickneswaran M, Duraisamy S, Atan M. Coagulopathy in acute head injury--a study of its role as a prognostic indicator. Br J Neurosurg 1997; 11:398.
  26. Gilbert WM, Danielsen B. Amniotic fluid embolism: decreased mortality in a population-based study. Obstet Gynecol 1999; 93:973.
  27. Sibai BM, Ramadan MK, Usta I, et al. Maternal morbidity and mortality in 442 pregnancies with hemolysis, elevated liver enzymes, and low platelets (HELLP syndrome). Am J Obstet Gynecol 1993; 169:1000.
  28. Fourrier F, Chopin C, Goudemand J, et al. Septic shock, multiple organ failure, and disseminated intravascular coagulation. Compared patterns of antithrombin III, protein C, and protein S deficiencies. Chest 1992; 101:816.
  29. Toh CH, Alhamdi Y, Abrams ST. Current Pathological and Laboratory Considerations in the Diagnosis of Disseminated Intravascular Coagulation. Ann Lab Med 2016; 36:505.
  30. Di Nisio M, Thachil J, Squizzato A. Management of disseminated intravascular coagulation: a survey of the International Society on Thrombosis and Haemostasis. Thromb Res 2015; 136:239.
  31. Squizzato A, Hunt BJ, Kinasewitz GT, et al. Supportive management strategies for disseminated intravascular coagulation. An international consensus. Thromb Haemost 2016; 115:896.
  32. Warren BL, Eid A, Singer P, et al. Caring for the critically ill patient. High-dose antithrombin III in severe sepsis: a randomized controlled trial. JAMA 2001; 286:1869.
  33. Ratnoff OD. Epsilon aminocaproic acid--a dangerous weapon. N Engl J Med 1969; 280:1124.
  34. Kitchens CS. Thrombocytopenia and thrombosis in disseminated intravascular coagulation (DIC). Hematology Am Soc Hematol Educ Program 2009; :240.
  35. Dreyfus M, Magny JF, Bridey F, et al. Treatment of homozygous protein C deficiency and neonatal purpura fulminans with a purified protein C concentrate. N Engl J Med 1991; 325:1565.
  36. Rintala E, Seppälä OP, Kotilainen P, et al. Protein C in the treatment of coagulopathy in meningococcal disease. Crit Care Med 1998; 26:965.
  37. Schellongowski P, Bauer E, Holzinger U, et al. Treatment of adult patients with sepsis-induced coagulopathy and purpura fulminans using a plasma-derived protein C concentrate (Ceprotin). Vox Sang 2006; 90:294.
  38. Goldenberg NA, Manco-Johnson MJ. Protein C deficiency. Haemophilia 2008; 14:1214.
  39. Mesters RM, Mannucci PM, Coppola R, et al. Factor VIIa and antithrombin III activity during severe sepsis and septic shock in neutropenic patients. Blood 1996; 88:881.
  40. Stéphan F, Hollande J, Richard O, et al. Thrombocytopenia in a surgical ICU. Chest 1999; 115:1363.
  41. Olson JD, Kaufman HH, Moake J, et al. The incidence and significance of hemostatic abnormalities in patients with head injuries. Neurosurgery 1989; 24:825.
  42. García-Avello A, Lorente JA, Cesar-Perez J, et al. Degree of hypercoagulability and hyperfibrinolysis is related to organ failure and prognosis after burn trauma. Thromb Res 1998; 89:59.
  43. Yu M, Nardella A, Pechet L. Screening tests of disseminated intravascular coagulation: guidelines for rapid and specific laboratory diagnosis. Crit Care Med 2000; 28:1777.
  44. Levi M. Current understanding of disseminated intravascular coagulation. Br J Haematol 2004; 124:567.
  45. Bakhtiari K, Meijers JC, de Jonge E, Levi M. Prospective validation of the International Society of Thrombosis and Haemostasis scoring system for disseminated intravascular coagulation. Crit Care Med 2004; 32:2416.
  46. Gando S, Iba T, Eguchi Y, et al. A multicenter, prospective validation of disseminated intravascular coagulation diagnostic criteria for critically ill patients: comparing current criteria. Crit Care Med 2006; 34:625.
  47. Gando S, Saitoh D, Ogura H, et al. Natural history of disseminated intravascular coagulation diagnosed based on the newly established diagnostic criteria for critically ill patients: results of a multicenter, prospective survey. Crit Care Med 2008; 36:145.