Disseminated Intravascular Coagulation Differential Diagnoses

Updated: Sep 29, 2016
  • Author: Marcel M Levi, MD; Chief Editor: Srikanth Nagalla, MBBS, MS, FACP  more...
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DDx

Diagnostic Considerations

The differential diagnosis of disseminated intravascular coagulation (DIC) is broad and can include other causes of consumptive coagulopathies, such as trauma and major surgery. In addition, severe liver disease can result in markedly reduced production of coagulation factors and inhibitors. Thrombocytopenia may also occur in this setting secondary to splenic sequestration, resulting in an overall clinical picture quite similar to DIC.

Thrombotic thrombocytopenic purpura (TTP) – hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy superficially like DIC, but distinctly different; in contrast to DIC, the mechanism of thrombosis is not via the tissue factor (TF)/factor VIIa pathway. Results of blood coagulation assays in TTP-HUS are normal. Rather, in TTP-HUS, thrombosis arises from direct platelet activation, usually as a result of widespread endothelial damage or an inherited or acquired impairment of ADAMTS13, a protease that normally cleaves von Willebrand factor (vWF), which results in an ultralarge vWF (ULVWF) that agglutinates/activates platelets, leading to thrombosis and shearing of red blood cells on the ULVWF.

TTP-HUS and DIC can usually be distinguished on the basis of their occurrence in different clinical settings (ie, trauma or sepsis for DIC and fever associated with thrombocytopenia and a microangiopathic hemolytic anemia for TTP-HUS). Patients with TTP-HUS do not demonstrate the laboratory abnormalities frequently encountered in DIC (see Workup). [43]

Other thrombotic microangiopathies include chemotherapy-induced or stem cell transplant–associated microangiopathy and HIV-induced TTP. [44]

Thrombocytopenia is present in both DIC and immune thrombocytopenic purpura (ITP). However, ITP is distinct from DIC in terms of its pathophysiologic mechanism and does not involve coagulation activation or microangiopathic hemolytic anemia.

Heparin-induced thrombocytopenia and thrombosis syndrome (HITTS) is another clinical entity with a presentation similar to that of DIC. A subpopulation of patients who have received heparin develop antibodies against platelet antigens (PF4), and a diminution of platelet number can result. If the antibody is particularly reactive with platelets, its direct activation of platelets leads to thrombosis. In HITTS, although the platelet count is decreased, the plasma prothrombin time (PT), the activated partial thromboplastin time (aPTT), and the fibrinogen levels are normal. Thrombosis is observed, but, typically, HITTS does not produce the consumptive coagulopathy of DIC.

Differential Diagnoses