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081999 Disseminated Intravascular Coagulation The New England Journal of Medicine Current Concepts Systemic activation+ of coagulation DISSEMINATED INTRAVASCULAR COAGULATION Intravascular+ Depletion of platelets+ deposition of fibrin and coagulation factors MARCEL LEVI, M.D., AND HUGO TEN CATE, M.D. Thrombosis of small+ Bleeding and midsize vessels+ ISSEMINATED intravascular coagulation is and organ failure characterized by the widespread activation Dof coagulation, which results in the intravas- Figure 1. The Mechanism of Disseminated Intravascular Coag- cular formation of fibrin and ultimately thrombotic ulation. occlusion of small and midsize vessels.1-3 Intravascu- Systemic activation of coagulation leads to widespread intra- lar coagulation can also compromise the blood sup- vascular deposition of fibrin and depletion of platelets and co- agulation factors. As a result, thrombosis of small and midsize ply to organs and, in conjunction with hemodynam- vessels may occur, contributing to organ failure, and there may ic and metabolic derangements, may contribute to be severe bleeding. the failure of multiple organs. At the same time, the use and subsequent depletion of platelets and coag- ulation proteins resulting from the ongoing coagu- lation may induce severe bleeding (Fig. 1). Bleeding may be the presenting symptom in a patient with disseminated intravascular coagulation, a factor that can complicate decisions about treatment. TABLE 1. COMMON CLINICAL CONDITIONS ASSOCIATED WITH DISSEMINATED ASSOCIATED CLINICAL CONDITIONS INTRAVASCULAR COAGULATION. AND INCIDENCE Sepsis Infectious Disease Trauma Serious tissue injury Disseminated intravascular coagulation is an ac- Head injury Fat embolism quired disorder that occurs in a wide variety of clin- Cancer ical conditions, the most important of which are listed Myeloproliferative diseases in Table 1. Infectious disease, in particular septicemia, Solid tumors (e.g., pancreatic carcinoma, prostatic carcinoma) is the most common clinical condition associated Obstetrical complications with disseminated intravascular coagulation. Although Amniotic-fluid embolism virtually all microorganisms can cause disseminated Abruptio placentae Vascular disorders intravascular coagulation, bacterial infection is most Giant hemangioma (Kasabach–Merritt syndrome) frequently related to the development of the syn- Aortic aneurysm Reactions to toxins (e.g., snake venom, drugs, am- drome. Clinically overt disseminated intravascular co- phetamines) agulation may occur in 30 to 50 percent of patients Immunologic disorders with gram-negative sepsis.4-6 Contrary to widely held Severe allergic reaction Hemolytic transfusion reaction belief, clinically overt disseminated intravascular co- Transplant rejection agulation appears to be as common in patients with gram-positive sepsis as in those with gram-negative sepsis.7 Triggers of the activation of diffuse coagula- tion in patients with infections are cell-specific mem- brane components of the microorganism, such as From the Department of Vascular Medicine and Internal Medicine, Ac- lipopolysaccharide or endotoxin, or bacterial exo- ademic Medical Center, University of Amsterdam (M.L., H.C.), and the De- partment of Internal Medicine, Slotervaart Hospital (H.C.) — both in Am- toxins (e.g., staphylococcal a hemolysin). All these sterdam. Address reprint requests to Dr. Levi at the Department of Vascular components may induce a generalized inflammatory Medicine and Internal Medicine, Academic Medical Centre F-4, Meiberg- dreef 9, 1105 AZ Amsterdam, the Netherlands, or at [email protected]. response, characterized by the activation of the cy- ©1999, Massachusetts Medical Society. tokine network. 586 · August 19, 1999 CURRENT CONCEPTS Severe Trauma Giant Hemangiomas Another clinical condition frequently associated Giant hemangiomas (the Kasabach–Merritt syn- with disseminated intravascular coagulation is severe drome) and even large aortic aneurysms may result trauma, particularly to the brain.1,6,8 A combination in local activation of coagulation.17,18 In patients with of mechanisms, including the release of fat and phos- these conditions, local activation of coagulation most pholipids from tissue into the circulation, hemolysis, commonly results in the systemic depletion of local- and endothelial damage, may promote the systemic ly consumed coagulation factors and platelets, but activation of coagulation. In addition, there is emerg- activated coagulation factors can reach the systemic ing evidence that cytokines have a pivotal role in the circulation and cause disseminated intravascular co- development of disseminated intravascular coagu- agulation. The incidence of clinically overt dissemi- lation, since the systemic activation patterns of cy- nated intravascular coagulation is 25 percent among tokines are virtually identical in patients with poly- patients with giant hemangiomas, whereas the inci- trauma and patients with sepsis.9,10 The incidence of dence is approximately 0.5 to 1 percent among pa- disseminated intravascular coagulation among patients tients with large aortic aneurysms.18,19 with severe trauma who have a systemic inflammato- ry response syndrome as a consequence is 50 to 70 Microangiopathic Hemolytic Anemia percent.6,9 Microangiopathic hemolytic anemias comprise thrombocytopenic thrombotic purpura, the hemolyt- Cancer ic–uremic syndrome, chemotherapy-induced micro- Both solid tumors and hematologic cancers may angiopathic hemolytic anemia, malignant hyperten- be complicated by disseminated intravascular coagu- sion, and the HELLP syndrome (hemolysis, elevated lation. Ten to 15 percent of patients with metasta- liver-enzyme levels, and a low platelet count occur- sized tumors have evidence of disseminated intravas- ring in association with preeclampsia).20 Although cular coagulation, and the condition is present in some characteristics of microangiopathic hemolytic approximately 15 percent of patients with acute leu- anemias and the resulting thrombotic occlusion of kemia.11,12 The mechanism of the derangement of small and midsize vessels may mimic the clinical pic- the coagulation system in patients with cancer is not ture of disseminated intravascular coagulation, these clear. However, a number of studies indicate that tis- disorders are a distinct group of diseases. The com- sue factor, which is expressed on the surface of tumor mon feature of the microangiopathic hemolytic ane- cells, is involved.13 A distinct form of disseminated mias appears to be endothelial damage, which causes intravascular coagulation is frequently encountered the adhesion and aggregation of platelets, the forma- in patients with acute promyelocytic leukemia, which tion of thrombin, and the impairment of fibrinolysis. is characterized by a severe hyperfibrinolytic state In patients with thrombocytopenic thrombotic pur- in addition to an activated coagulation system. Al- pura and the hemolytic–uremic syndrome, the ac- though bleeding is the most common clinical fea- quired deficiency of a protease that cleaves multimers ture, disseminated thrombosis is found in some pa- of von Willebrand factor leads to the accumulation tients at autopsy.14 Treatment with all-trans-retinoic of large multimers of this factor.21 A cardinal sign of acid, however, has drastically reduced the incidence microangiopathic hemolytic anemia is the presence of severe disseminated intravascular coagulation in of fragmented red cells (schistocytes) in the blood patients with acute promyelocytic leukemia. smear. Although hemolytic anemia and schistocytes are also sometimes present in patients with severe Obstetrical Disorders disseminated intravascular coagulation (because of Disseminated intravascular coagulation is a classic the presence of intravascular fibrin), these are invari- complication of obstetrical conditions, such as abrup- able findings in patients with microangiopathic hemo- tio placentae and amniotic-fluid embolism, occur- lytic anemia. ring in more than 50 percent of patients with these conditions.15 Leakage of thromboplastin-like material CLINICAL RELEVANCE AND PROGNOSIS is likely to cause the systemic activation of coagula- The contribution of disseminated intravascular co- tion, since amniotic fluid is a potent activator of co- agulation to morbidity and the risk of mortality var- agulation in vitro and since the degree of placental ies depending on the underlying clinical condition separation correlates with the extent of disseminated and the intensity of the coagulation disorder. Ob- intravascular coagulation. Although these obstetrical viously, the seriousness of a severe depletion of conditions may cause fulminant disseminated intra- platelets and coagulation factors in patients with dif- vascular coagulation, the disorder is usually short-lived fuse and ongoing bleeding or in patients at high risk and self-limiting. Preeclampsia can also be compli- for bleeding (e.g., patients recovering from surgery) cated by disseminated intravascular coagulation, which is indisputable. In addition, several lines of evidence occurred in 7 percent of consecutive patients with indicate that disseminated intravascular coagulation severe preeclampsia in one study.16 increases the risk of organ failure and death. First, Volume 341 Number 8 · 587 The New England Journal of Medicine histologic studies have shown that signs of ischemia Defects in Inhibitors of Coagulation and necrosis are related to the deposition of fibrin in All major physiologic anticoagulants — antithrom- the vasculature of organs in patients with dissemi-
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