Introduction
A quick literature search in the MEDLINE databases from 1966 to 1998 using the search
term disseminated intravascular coagulation (DIC) and related key words yields an
impressive 11,921 manuscripts. Most of the published literature concerns the pathophysiology
of DIC, which in its main features is now well understood. Other aspects of DIC, however,
particularly those related to the definition, the relevance of the syndrome, and clinical
management, remain unclear. Taking an evidence-based approach to the appropriate diagnosis
and treatment of patients with DIC is difficult, in view of the lack of sound clinical
trials. This is probably due to the fact that DIC is a poorly-defined syndrome with
a widely variable intensity, often complicating a diversity of severe disorders that
are themselves related to extensive morbidity and mortality.1,2 This chapter briefly reviews the clinical setting, incidence, and relevance of DIC
and current insights into the pathogenesis of DIC. It also discusses the available
knowledge on the clinical management of patients with this syndrome.
DIC is not a disease or a symptom but rather a syndrome, which is always secondary
to an underlying disorder. The syndrome is characterized by a systemic activation
of the blood coagulation system, which results in the generation and deposition of
fibrin, leading to microvascular thrombi in various organs and contributing to the
development of multiorgan failure. Consumption and subsequent exhaustion of coagulation
proteins and platelets, due to the ongoing activation of the coagulation system, may
induce severe bleeding complications, although microclot formation may occur in the
absence of severe clotting factor depletion and bleeding.3 Derangement of the fibrinolytic system further contributes to intravascular clot
formation (discussed later), but in some cases accelerated fibrinolysis (e.g., due
to consumption of α2-antiplasmin) may cause severe bleeding. Hence, a patient with DIC can present with
simultaneous thrombotic and bleeding problems, which obviously complicates treatment.
Although there is no general consensus regarding the definition of DIC, the definition
as put forward by Müller-Berghaus and colleagues in 1995 might be most appropriate:
“Disseminated intravascular coagulation is an acquired syndrome characterized by the
activation of intravascular coagulation up to intravascular fibrin formation. The
process may be accompanied by secondary fibrinolysis or inhibited fibrinolysis.”4
