Disseminated Intravascular Coagulation

  • Mario V. Fusaro
  • Giora NetzerEmail author


A 64-year old woman with a history of non-small cell lung cancer, Type 2 diabetes, and hypertension presented to the emergency department with altered mental status and flank pain. In the emergency department, her initial blood pressure was 83/44 mmHg, with a pulse of 110 beats per minute. On physical examination, she was somnolent but arousable. She had a fine petechial rash on both lower extremities and bleeding from her peripheral IV sites. Laboratory testing results included: white blood cell count of 3100/μL, creatinine of 2.5 mg/dL (her baseline being 1.0), International Normalized Ratio (INR) of 3.1, Partial Thromboplastin Time of 50 s, and platelet count of 33,000 cells/μL. The urine microscopy revealed 20–50 WBC/high-powered field; the urine culture is growing 100,000 colony-forming units of a gram negative bacillus. A computerized tomography (CT) scan of the abdomen and pelvis revealed left hydronephrosis, hydroureter, and perinephric stranding. A slide of the patient’s peripheral blood smear is shown in the Fig. 78.1.


  1. 1.
    Singh B, Hanson AC, Alhurani R, Wang S, Herasevich V, Cartin-Ceba R, et al. Trends in the incidence and outcomes of disseminated intravascular coagulation in critically ill patients (2004–2010): a population-based study. Chest. 2013;143(5):1235–42.CrossRefGoogle Scholar
  2. 2.
    Gando S, Saitoh D, Ogura H, Mayumi T, Koseki K, Ikeda T, 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(1):145–50.CrossRefGoogle Scholar
  3. 3.
    Smith OP, White B. Infectious purpura fulminans: diagnosis and treatment. Br J Haematol. 1999;104(2):202–7.CrossRefGoogle Scholar
  4. 4.
    Schmaier AH. Disseminated intravascular coagulation. N Engl J Med. 1999;341(25):1937–8.PubMedGoogle Scholar
  5. 5.
    Laursen MA, Larsen JB, Hvas AM. Platelet function in disseminated intravascular coagulation: a systematic review. Platelets. 2018;29(3):238–48.CrossRefGoogle Scholar
  6. 6.
    Kitchens CS. Thrombocytopenia and thrombosis in disseminated intravascular coagulation (DIC). Hematology Am Soc Hematol Educ Program. 2009:240–6.Google Scholar
  7. 7.
    Toh CH, Hoots WK, SSC on Disseminated Intravascular Coagulation of the ISTH. The scoring system of the Scientific and Standardisation Committee on Disseminated Intravascular Coagulation of the International Society on Thrombosis and Haemostasis: a 5-year overview. J Thromb Haemost. 2007;5(3):604–6.CrossRefGoogle Scholar
  8. 8.
    Lee DH, Lee BK, Jeung KW, Park JS, Lim YD, Jung YH, et al. Performance of 5 disseminated intravascular coagulation score systems in predicting mortality in patients with severe trauma. Medicine (Baltimore). 2018;97(33):e11912.CrossRefGoogle Scholar
  9. 9.
    Wada H, Gabazza EC, Asakura H, Koike K, Okamoto K, Maruyama I, et al. Comparison of diagnostic criteria for disseminated intravascular coagulation (DIC): diagnostic criteria of the International Society of Thrombosis and Hemostasis and of the Japanese Ministry of Health and Welfare for overt DIC. Am J Hematol. 2003;74(1):17–22.CrossRefGoogle Scholar
  10. 10.
    Iba T, Nisio MD, Levy JH, Kitamura N, Thachil J. New criteria for sepsis-induced coagulopathy (SIC) following the revised sepsis definition: a retrospective analysis of a nationwide survey. BMJ Open. 2017;7(9):e017046-2017-017046Google Scholar
  11. 11.
    Peigne V, Azoulay E, Coquet I, Mariotte E, Darmon M, Legendre P, et al. The prognostic value of ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13) deficiency in septic shock patients involves interleukin-6 and is not dependent on disseminated intravascular coagulation. Crit Care. 2013;17(6):R273.CrossRefGoogle Scholar
  12. 12.
    Wakabayashi I, Mambo N, Ueda T, Nonaka D, Lee LJ, Tanaka K, et al. New biomarkers for prediction of disseminated intravascular coagulation in patients with sepsis. Clin Appl Thromb Hemost. 2018. 1076029618804078;
  13. 13.
    Wada H, Matsumoto T, Suzuki K, Imai H, Katayama N, Iba T, et al. Differences and similarities between disseminated intravascular coagulation and thrombotic microangiopathy. Thromb J. 2018;16:14-018-0168-2. eCollection 2018Google Scholar
  14. 14.
    Levi M. Clinical characteristics of disseminated intravascular coagulation in patients with solid and hematological cancers. Thromb Res. 2018;164(Suppl 1):S77–81.CrossRefGoogle Scholar
  15. 15.
    Martin JN Jr, Blake PG, Lowry SL, Perry KG Jr, Files JC, Morrison JC. Pregnancy complicated by preeclampsia-eclampsia with the syndrome of hemolysis, elevated liver enzymes, and low platelet count: how rapid is postpartum recovery? Obstet Gynecol. 1990;76(5 Pt 1):737–41.CrossRefGoogle Scholar
  16. 16.
    Levi M, Sivapalaratnam S. Disseminated intravascular coagulation: an update on pathogenesis and diagnosis. Expert Rev Hematol. 2018;11(8):663–72.CrossRefGoogle Scholar
  17. 17.
    Gu WJ, Wang F, Bakker J, Tang L, Liu JC. The effect of goal-directed therapy on mortality in patients with sepsis—earlier is better: a meta-analysis of randomized controlled trials. Crit Care. 2014;18(5):570.CrossRefGoogle Scholar
  18. 18.
    Churchill D, Duley L, Thornton JG, Moussa M, Ali HS, Walker KF. Interventionist versus expectant care for severe pre-eclampsia between 24 and 34 weeks' gestation. Cochrane Database Syst Rev. 2018;10:CD003106.PubMedGoogle Scholar
  19. 19.
    Carson JL, Grossman BJ, Kleinman S, Tinmouth AT, Marques MB, Fung MK, et al. Red blood cell transfusion: a clinical practice guideline from the AABB∗. Ann Intern Med. 2012;157(1):49–58.CrossRefGoogle Scholar
  20. 20.
    Kaufman RM, Djulbegovic B, Gernsheimer T, Kleinman S, Tinmouth AT, Capocelli KE, et al. Platelet transfusion: a clinical practice guideline from the AABB. Ann Intern Med. 2015;162(3):205–13.CrossRefGoogle Scholar
  21. 21.
    Roback JD, Caldwell S, Carson J, Davenport R, Drew MJ, Eder A, et al. Evidence-based practice guidelines for plasma transfusion. Transfusion. 2010;50(6):1227–39.CrossRefGoogle Scholar
  22. 22.
    Abdel-Wahab OI, Healy B, Dzik WH. Effect of fresh-frozen plasma transfusion on prothrombin time and bleeding in patients with mild coagulation abnormalities. Transfusion. 2006;46(8):1279–85.CrossRefGoogle Scholar
  23. 23.
    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(1):24–33.CrossRefGoogle Scholar
  24. 24.
    Nishida T, Kinoshita T, Yamakawa K. Tranexamic acid and trauma-induced coagulopathy. J Intensive Care. 2017;5:5-016-0201-0. eCollection 2017Google Scholar
  25. 25.
    AABB, the American Red Cross, America’s Blood Centers, and the Armed Services Blood Program. Information Piece: Alternatives to Transfusable Single-Donor Plasma Components; 2014.Google Scholar
  26. 26.
    Lerolle N, Carlotti A, Melican K, Aubey F, Pierrot M, Diehl JL, et al. Assessment of the interplay between blood and skin vascular abnormalities in adult purpura fulminans. Am J Respir Crit Care Med. 2013;188(6):684–92.CrossRefGoogle Scholar
  27. 27.
    Wada H, Thachil J, Di Nisio M, Mathew P, Kurosawa S, Gando S, et al. Guidance for diagnosis and treatment of DIC from harmonization of the recommendations from three guidelines. J Thromb Haemost. 2013;
  28. 28.
    Schwameis M, Buchtele N, Schober A, Schoergenhofer C, Quehenberger P, Jilma B. Prognosis of overt disseminated intravascular coagulation in patients admitted to a medical emergency department. Eur J Emerg Med. 2017;24(5):340–6.CrossRefGoogle Scholar
  29. 29.
    Stephan F, Hollande J, Richard O, Cheffi A, Maier-Redelsperger M, Flahault A. Thrombocytopenia in a surgical ICU. Chest. 1999;115(5):1363–70.CrossRefGoogle Scholar
  30. 30.
    Kim J, Kim K, Lee JH, Jo YH, Kim T, Rhee JE, et al. Prognostic implication of initial coagulopathy in out-of-hospital cardiac arrest. Resuscitation. 2013;84(1):48–53.CrossRefGoogle Scholar
  31. 31.
    Westendorp RG, Hottenga JJ, Slagboom PE. Variation in plasminogen-activator-inhibitor-1 gene and risk of meningococcal septic shock. Lancet. 1999;354(9178):561–3.CrossRefGoogle Scholar
  32. 32.
    Pernerstorfer T, Hollenstein U, Hansen J, Knechtelsdorfer M, Stohlawetz P, Graninger W, et al. Heparin blunts endotoxin-induced coagulation activation. Circulation. 1999;100(25):2485–90.CrossRefGoogle Scholar
  33. 33.
    Liu XL, Wang XZ, Liu XX, Hao D, Jaladat Y, Lu F, et al. Low-dose heparin as treatment for early disseminated intravascular coagulation during sepsis: a prospective clinical study. Exp Ther Med. 2014;7(3):604–8.CrossRefGoogle Scholar
  34. 34.
    Zarychanski R, Abou-Setta AM, Kanji S, Turgeon AF, Kumar A, Houston DS, et al. The efficacy and safety of heparin in patients with sepsis: a systematic review and metaanalysis∗. Crit Care Med. 2015;43(3):511–8.CrossRefGoogle Scholar
  35. 35.
    Tamura K, Saito H, Asakura H, Okamoto K, Tagawa J, Hayakawa T, et al. Recombinant human soluble thrombomodulin (thrombomodulin alfa) to treat disseminated intravascular coagulation in solid tumors: results of a one-arm prospective trial. Int J Clin Oncol. 2015;20(4):821–8.CrossRefGoogle Scholar
  36. 36.
    Ogawa Y, Yamakawa K, Ogura H, Kiguchi T, Mohri T, Nakamori Y, et al. Recombinant human soluble thrombomodulin improves mortality and respiratory dysfunction in patients with severe sepsis. J Trauma Acute Care Surg. 2012;72(5):1150–7.CrossRefGoogle Scholar
  37. 37.
    Vincent JL, Ramesh MK, Ernest D, LaRosa SP, Pachl J, Aikawa N, et al. A randomized, double-blind, placebo-controlled, Phase 2b study to evaluate the safety and efficacy of recombinant human soluble thrombomodulin, ART-123, in patients with sepsis and suspected disseminated intravascular coagulation. Crit Care Med. 2013;41(9):2069–79.CrossRefGoogle Scholar
  38. 38.
    Kato T, Matsuura K. Recombinant human soluble thrombomodulin improves mortality in patients with sepsis especially for severe coagulopathy: a retrospective study. Thromb J. 2018;16:19-018-0172-6. eCollection 2018Google Scholar
  39. 39.
    Zhang C, Wang H, Yang H, Tong Z. Recombinant human soluble thrombomodulin and short-term mortality of infection patients with DIC: a meta-analysis. Am J Emerg Med. 2016;34(9):1876–82.CrossRefGoogle Scholar
  40. 40.
    Wiedermann CJ, Kaneider NC. A systematic review of antithrombin concentrate use in patients with disseminated intravascular coagulation of severe sepsis. Blood Coagul Fibrinolysis. 2006;17(7):521–6.CrossRefGoogle Scholar
  41. 41.
    Gando S, Saitoh D, Ishikura H, Ueyama M, Otomo Y, Oda S, et al. A randomized, controlled, multicenter trial of the effects of antithrombin on disseminated intravascular coagulation in patients with sepsis. Crit Care. 2013;17(6):R297.CrossRefGoogle Scholar
  42. 42.
    Hayakawa M, Kudo D, Saito S, Uchino S, Yamakawa K, Iizuka Y, et al. Antithrombin supplementation and mortality in sepsis-induced disseminated intravascular coagulation: a multicenter retrospective observational study. Shock. 2016;46(6):623–31.CrossRefGoogle Scholar
  43. 43.
    Yatabe T, Inoue S, Sakamoto S, Sumi Y, Nishida O, Hayashida K, et al. The anticoagulant treatment for sepsis induced disseminated intravascular coagulation; network meta-analysis. Thromb Res. 2018;171:136–42.CrossRefGoogle Scholar
  44. 44.
    Yasuda N, Goto K, Ohchi Y, Abe T, Koga H, Kitano T. The efficacy and safety of antithrombin and recombinant human thrombomodulin combination therapy in patients with severe sepsis and disseminated intravascular coagulation. J Crit Care. 2016;36:29–34.CrossRefGoogle Scholar
  45. 45.
    Iba T, Hagiwara A, Saitoh D, Anan H, Ueki Y, Sato K, et al. Effects of combination therapy using antithrombin and thrombomodulin for sepsis-associated disseminated intravascular coagulation. Ann Intensive Care. 2017;7(1):110-017-0332-zGoogle Scholar
  46. 46.
    Umemura Y, Yamakawa K, Hayakawa M, Kudo D, Fujimi S. Concomitant versus individual administration of antithrombin and thrombomodulin for sepsis-induced disseminated intravascular coagulation: A Nationwide Japanese Registry Study. Clin Appl Thromb Hemost. 2018;24(5):734–40.CrossRefGoogle Scholar

Copyright information

© This is a U.S. government work and not under copyright protection in the U.S.; foreign copyright protection may apply 2020

Authors and Affiliations

  1. 1.Department of MedicineWestchester Medical CenterValhallaUSA
  2. 2.Division of Pulmonary and Critical Care Medicine, Department of Epidemiology and Public HealthUniversity of MarylandBaltimoreUSA

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