Disseminated intravascular coagulation

Disseminated intravascular coagulation (DIC) is an acquired syndrome due to an underlying disease or pathologic process that initially activates coagulation, resulting in a consumptive coagulopathy with depletion of platelets and coagulation factors, and microvascular thrombosis.

The most common cause of DIC is sepsis, but other clinical causes include shock with multiorgan failure, obstetric causes, cancer, and major tissue injury. Clinical presentation can also include either bleeding or thrombosis. Physical exam may reveal petechiae, ecchymosis, acrocyanosis, symmetric peripheral gangrene, mental status changes, hypoxemia, hypotension, venous thrombosis, and bleeding.

Diagnosis is based on the presence of ≥1 known underlying conditions causing DIC plus abnormal global coagulation tests: thrombocytopenia, increased prothrombin time/international normalized ratio, elevated fibrin-related marker (D-dimer/fibrin degradation products), and often hypofibrinogenemia.

Active treatment of the underlying disorder is the mainstay of therapy. With sepsis being the major cause, early and timely intervention with antimicrobial therapy is critical. Treatment of DIC commonly requires acute cardiopulmonary support and potentially coagulopathy treatment depending on presentation (hypercoagulable or hemorrhagic) with fresh frozen plasma, platelets, and anticoagulation.

In addition to coagulopathy, multiorgan failure can occur with, for example, acute renal failure or acute lung injury, and acrocyanosis with loss of digits.

DIC is an acquired, life-threatening intravascular disorder characterized by systemic coagulation activation, dysregulated fibrinolysis, and endothelial injury, resulting in microthrombosis. DIC arises from various underlying etiologies and progresses from a potentially asymptomatic early phase to an advanced phase with hemorrhage and/or organ dysfunction.[1]Iba T, Levy JH, Maier CL, et al. Updated definition and scoring of disseminated intravascular coagulation in 2025: communication from the ISTH SSC subcommittee on disseminated intravascular coagulation. J Thromb Haemost. 2025 Jul;23(7):2356-62. https://www.jthjournal.org/article/S1538-7836(25)00220-X/fulltext http://www.ncbi.nlm.nih.gov/pubmed/40216223?tool=bestpractice.com

DIC is most commonly a complication of acute infections/sepsis. It also occurs due to shock, obstetric disorders, malignancies, vascular injury, trauma, and toxic or immunologic reactions and organ injury.[2]Squizzato A, Gallo A, Levi M, et al. Underlying disorders of disseminated intravascular coagulation: communication from the ISTH SSC subcommittees on disseminated intravascular coagulation and perioperative and critical care thrombosis and hemostasis. J Thromb Haemost. 2020 Sep;18(9):2400-7. https://www.jthjournal.org/article/S1538-7836(22)01650-6/fulltext http://www.ncbi.nlm.nih.gov/pubmed/32881338?tool=bestpractice.com [3]Iba T, Helms J, Connors JM, et al. The pathophysiology, diagnosis, and management of sepsis-associated disseminated intravascular coagulation. J Intensive Care. 2023 May 23;11(1):24. https://pmc.ncbi.nlm.nih.gov/articles/PMC10202753 http://www.ncbi.nlm.nih.gov/pubmed/37221630?tool=bestpractice.com In coronavirus disease 2019 (COVID-19) associated coagulopathy, the initial presentation is a hypercoagulable state with elevated fibrinogen and D-dimers, with normal prothrombin times and platelet counts. However, with ongoing hospitalization, secondary infections are common with the potential for subsequently developing DIC.