title: Disseminated intravascular coagulation.
abstract: Disseminated intravascular coagulation (DIC) is an acquired syndrome characterized by widespread intravascular activation of coagulation that can be caused by infectious insults (such as sepsis) and non-infectious insults (such as trauma). The main pathophysiological mechanisms of DIC are inflammatory cytokine-initiated activation of tissue factor-dependent coagulation, insufficient control of anticoagulant pathways and plasminogen activator inhibitor 1-mediated suppression of fibrinolysis. Together, these changes give rise to endothelial dysfunction and microvascular thrombosis, which can cause organ dysfunction and seriously affect patient prognosis. Recent observations have pointed to an important role for extracellular DNA and DNA-binding proteins, such as histones, in the pathogenesis of DIC. The International Society on Thrombosis and Haemostasis (ISTH) established a DIC diagnostic scoring system consisting of global haemostatic test parameters. This scoring system has now been well validated in diverse clinical settings. The theoretical cornerstone of DIC management is the specific and vigorous treatment of the underlying conditions, and DIC should be simultaneously managed to improve patient outcomes. The ISTH guidance for the treatment of DIC recommends treatment strategies that are based on current evidence. In this Primer, we provide an updated overview of the pathophysiology, diagnosis and management of DIC and discuss the future directions of basic and clinical research in this field.
authors.affiliation: Department of Anesthesiology and Critical Care Medicine, Hokkaido University Graduate School of Medicine, N15W7, Kita-ku, Sapporo, 060-8638, Japan.
authors.forename: Satoshi
authors.initials: S
authors.lastname: Gando
authors.affiliation: Department of Medicine, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands.
authors.forename: Marcel
authors.initials: M
authors.lastname: Levi
authors.affiliation: Institute of Infection and Global Health, University of Liverpool, Liverpool, UK.
authors.forename: Cheng-Hock
authors.initials: CH
authors.lastname: Toh
mesh_terms: D000933:Antifibrinolytic Agents / Q000493:pharmacokinetics / Q000494:pharmacology / Q000627:therapeutic use; D001777:Blood Coagulation / Q000502:physiology; D004211:Disseminated Intravascular Coagulation / Q000150:complications* / Q000453:epidemiology / Q000503:physiopathology*; D005172:Factor XI / Q000032:analysis / Q000502:physiology; D005173:Factor XI Deficiency / Q000097:blood / Q000503:physiopathology; D005342:Fibrinolysis / Q000276:immunology / Q000502:physiology; D006801:Humans; D011379:Prognosis; D011486:Protein C / Q000032:analysis / Q000502:physiology; D020151:Protein C Deficiency / Q000097:blood / Q000503:physiopathology; D018805:Sepsis / Q000209:etiology; D018746:Systemic Inflammatory Response Syndrome / Q000150:complications; D013927:Thrombosis / Q000209:etiology
chemical_list: D000933:Antifibrinolytic Agents; D011486:Protein C; D005172:Factor XI