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To review the current knowledge on the clinical manifestation, pathogenesis, diagnosis, and management of disseminated intravascular coagulation (DIC).Selected articles from the MEDLINE database.DIC may complicate a variety of disorders and can cause significant morbidity (in particular related to organ dysfunction and bleeding) and may contribute to mortality. The pathogenesis of DIC is based on tissue factor–mediated initiation of systemic coagulation activation that is insufficiently contained by physiologic anticoagulant pathways and amplified by impaired endogenous fibrinolysis. The diagnosis of DIC can be made using routinely available laboratory tests and scoring algorithms. Supportive treatment of DIC may be aimed at replacement of platelets and coagulation factors, anticoagulant treatment, and restoration of anticoagulant pathways.Insight into the pathogenesis of DIC has resulted in better strategies for clinical management, including straightforward diagnostic criteria and potentially beneficial supportive treatment options.