Disseminated intravascular coagulation and coagulation disorders

Abstract

An update on recent developments in diagnosis and treatment of disseminated intravascular coagulation. Disseminated intravascular coagulation is defined as a typical disease condition with laboratory findings indicating massive coagulation activation and reduction in procoagulant capacity. Clinical syndromes associated with the condition are consumption coagulopathy, sepsis-induced purpura fulminans, and viral hemorrhagic fevers. Consumption coagulopathy is observed in patients with sepsis, aortic aneurysms, acute promyelocytic leukemia, and other disseminated malignancies. Sepsis-induced purpura fulminans is characterized by microvascular occlusion causing hemorrhagic necrosis of the skin and organ failure. Viral hemorrhagic fevers result in massively increased tissue factor production in monocytes and macrophages, inducing microvascular thrombosis and consumption of platelets and coagulation factors. Current scoring systems do not distinguish between patients with asymptomatic disseminated intravascular coagulation, consumption coagulopathy and thrombotic syndromes. Patients with sepsis may be identified by activated partial thromboplastin time waveform analysis performed as part of routine coagulation testing. Drotrecogin alpha (activated) reduces mortality in patients with severe sepsis with and without disseminated intravascular coagulation and has been used in patients with sepsis-induced purpura fulminans. Tifacogin does not reduce mortality in severe sepsis associated with impaired coagulation. Patients with heterozygous factor V Leiden mutation and severe sepsis showed a lower 28-day mortality than patients without this mutation, supporting the assumption that an enhanced level of coagulation activation may be beneficial in patients with severe sepsis. Whereas antithrombin and tifacogin failed to improve clinical outcome in severe sepsis, drotrecogin alpha (activated) increased the chances of survival of patients with severe sepsis with and without disseminated intravascular coagulation.