Many diseases or disorders can lead to disseminated intravascular coagulation (DIC), as discussed in the previous article.
DIC can manifest in a variety of forms depending on the severity and duration of the procoagulant stimulus, so patients with
DIC can have a variety of signs. In addition, a gold standard test for establishing a definitive diagnosis is lacking, so
clinicians rely heavily on evaluating patients and appropriately interpreting available laboratory test results.
Treatment is challenging as well, because the primary disorder stimulating coagulation must be identified and eliminated for
a complete recovery. Supportive treatment of DIC consists of controlling excessive intravascular coagulation, maintaining
organ perfusion, and replacing coagulation components, if necessary. Supportive treatment is continued until the primary disease
can be eliminated. In this article, we provide guidance to help you identify and effectively treat patients with DIC early,
improving their outcome.
Because of its pathophysiology, DIC can manifest as massive thrombosis, hemorrhage, or both. Most often thrombosis and hemorrhage
occur simultaneously. Adding to this complexity, three clinical forms exist: peracute, acute (fulminant or end-stage), and
chronic (subclinical or low-grade).1-7 The varying forms seem to be related to the intensity of the procoagulant stimulus. For example, acute DIC may be a true
acute process or, more commonly, an acute decompensation of peracute DIC. Chronic DIC tends to be common with malignancies
and other localized or chronic, low-intensity hypercoagulable disorders (e.g. pulmonary thromboembolism).
Patients with peracute DIC most often demonstrate organ thrombosis with a mild consumptive thrombocytopenia. Thrombosis is
evidenced by clinical and clinicopathologic abnormalities indicative of organ dysfunction (Table 1). The most common organs or systems to thrombose are the kidneys, lungs, heart, central nervous system, and gastrointestinal
tract.2,6 These patients may present with a primary hemostatic abnormality; however, typically thrombocytopenia is mild, and no hemostatic
abnormalities are evident. Clotting times may be faster than normal and may be indicative of hypercoagulation.8 However, assessing D-dimer concentration is a more sensitive test for acute thromboembolism.9 The severity of organ failure determines these patients' outcomes.
Table 1. Common Sites of Thrombosis and the Respective Clinical Signs and Clinicopathologic Findings
Patients with acute DIC typically present with both thrombosis and hemorrhage. Acute DIC is typically an acute decompensation
of peracute DIC.8 The decompensation is secondary to ongoing damaged endothelium, damaged extravascular tissues, and increases in factor XII
with secondary excessive plasmin generation. Excessive plasmin causes rapid dissolution of fibrin clots and degradation of
factors V, VIII, IX, and XI. Primary (petechiation, ecchymosis, mucosal bleeding) and secondary (body cavity hemorrhage) hemostatic
abnormalities are present.1,2,4 Acute DIC is rapidly fatal, usually because of severe hypotension and irreversible shock.1,2,4
Clinical signs are less obvious and slow to develop in patients with chronic DIC. As a result, the body can compensate with
increased production of clotting factors, platelets, and inhibitors.1-4 Platelets tend to be decreased while coagulation factors are normal because bone marrow production (platelets) is more limited
than hepatic production (coagulation factors).7 This compensation prevents the spontaneous hemorrhage evident with acute DIC. However, if hemorrhage is evident, it is generally
due to primary hemostatic dysfunction. If recognized early, chronic DIC tends to have a better prognosis because multiple
organ dysfunction and severe shock are usually not present.