Syndromes of disseminated intravascular coagulation in obstetrics, pregnancy, and gynecology. Objective criteria for diagnosis and management.

Disseminated intravascular coagulation (DIC) is a confusing syndrome, because (1) many unrelated diseases can induce DIC, (2) clinical manifestations can vary, (3) there is confusion regarding appropriate laboratory diagnosis, and (4) the guidelines for management with specific available therapeutic modalities are unclear. Recommendations for and evaluation of management become even more difficult because the morbidity and survival often depends on the specific cause of DIC, and few of the generally used specific modes of therapy (heparin, antithrombin concentrate, protein C concentrate, and others) have been subjected to objective, prospective, randomized trials. This article provides a general review of the origin and pathophysiology of DIC in obstetric and gynecologic settings. It gives specific, objective guidelines and criteria for the clinical and laboratory diagnosis of DIC and objective systems for assessing the efficacy of any given specific therapeutic modality (independent of the influences of the underlying disease causing the DIC) in obstetric, pregnant, or gynecologic patients.15 This approach allows objective decisions to be made regarding diagnosis and management. Disseminated intravascular coagulation is an intermediary mechanism of disease usually seen in association with well-defined clinical disorders.9, 16, 17, 18 In pregnant women, those disorders include obstetric accidents Amniotic fluid embolism Placental abruption Placenta previa Pre-eclampsia Eclampsia Hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome Retained fetus syndrome Retained products of conception Abortion Disseminated intravascular coagulation can be associated with gynecologic malignancy: Ovarian cancer Uterine cancer Breast cancer Paraneoplastic syndromes As discussed later, DIC in obstetric and gynecologic patients may also develop from other medical and surgical complications not unique to obstetrics, pregnancy, or gynecology. Disseminated intravascular coagulation is an intermediary mechanism in many disease processes, which sometimes remain organ-specific. This catastrophic syndrome spans all areas of medicine and presents a broad clinical spectrum that is often confusing. In the early literature, DIC was called consumptive coagulopathy131, 198; this term is no longer regarded as a proper description, because very little is consumed in DIC; most factors and plasma constituents are plasmin-biodegraded. Later the term defibrination syndrome was used. The current designation, disseminated intravascular coagulation, is a useful descriptive pathophysiologic term if coagulation is understood to encompass both hemorrhage and thrombosis.9, 15, 16, 17, 18 Most physicians consider DIC to be a systemic hemorrhagic syndrome, because hemorrhage is obvious and often impressive. Less commonly appreciated is the formidable microvascular thrombosis and, sometimes, large-vessel thrombosis that occurs. Treating hemorrhage in patients with fulminant DIC is often simple; it is the small- and large-vessel thrombosis, with impaired blood flow, ischemia, and associated end-organ damage, that usually leads to irreversible morbidity and mortality. Throughout this article, fulminant DIC is contrasted with low-grade compensated DIC, and the attendant differences in clinical manifestations, laboratory findings and treatment are discussed. These distinctions, however, are often pure and theoretical. Clinically, there is a spectrum of DIC. Patients may present anywhere in this continuum, and the disease may progress from one end of the spectrum to the other. The minimal acceptable criteria for a diagnosis of DIC are a systematic thrombohemorrhagic disorder seen in association with well-defined clinical situations and laboratory evidence of 1Procoagulant activation 2Fibrinolytic activation 3Inhibitor consumption 4Biochemical evidence of end-organ damage or failure