Disseminated Intravascular Coagulation

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Chapter 477 Disseminated Intravascular Coagulation

J. Paul Scott, Leslie J. Raffini, Robert R. Montgomery

Thrombotic microangiopathy refers to a heterogeneous group of conditions, including disseminated intravascular coagulation (DIC), that result in consumption of clotting factors, platelets, and anticoagulant proteins. Consequences of this process include widespread intravascular deposition of fibrin, leading to tissue ischemia and necrosis, a generalized hemorrhagic state, and hemolytic anemia.

Etiology

Any life-threatening severe systemic disease associated with hypoxia, acidosis, tissue necrosis, shock, and/or endothelial damage may trigger DIC. A large number of conditions have been reported to be associated with DIC (Table 477-1). Although the clinical symptoms are more often hemorrhagic, the initiating event is usually excessive activation of clotting that consumes both the physiologic anticoagulants (protein C, protein S, and antithrombin III) and procoagulants, resulting in a deficiency of factor V, factor VIII, prothrombin, fibrinogen, and platelets. Commonly, the clinical result of this sequence of events is hemorrhage. The hemostatic dysregulation may also result in thromboses in the skin, kidneys, and other organs. Better understanding of the pathophysiology of hemostasis has lead to an appreciation of the critical interaction of the coagulation pathways with the innate immune system and inflammatory response that likely contributes to the widespread dysregulation present in DIC.

Table 477-1 CAUSES OF DISSEMINATED INTRAVASCULAR COAGULATION

INFECTIOUS

Meningococcemia (purpura fulminans)

Bacterial sepsis (staphylococcal, streptococcal, Escherichia coli, Salmonella)

Rickettsia (Rocky Mountain spotted fever)

Virus (cytomegalovirus, herpes simplex, hemorrhagic fevers)

Malaria

Fungus

TISSUE INJURY

Central nervous system trauma (massive head injury)

Multiple fractures with fat emboli

Crush injury

Profound shock or asphyxia

Hypothermia or hyperthermia

Massive burns

MALIGNANCY

Acute promyelocytic leukemia

Acute monoblastic or promyelocytic leukemia

Widespread malignancies (neuroblastoma)

VENOM OR TOXIN

Snake bites

Insect bites

MICROANGIOPATHIC DISORDERS

“Severe” thrombotic thrombocytopenic purpura or hemolytic-uremic syndrome

Giant hemangioma (Kasabach-Merritt syndrome)

GASTROINTESTINAL DISORDERS

Fulminant hepatitis

Severe inflammatory bowel disease

Pancreatitis

HEREDITARY THROMBOTIC DISORDERS

Antithrombin III deficiency

Homozygous protein C deficiency

NEWBORN

Maternal toxemia

Bacterial or viral sepsis (group B streptococcus, herpes simplex)

Abruptio placentae

Severe respiratory distress syndrome

Necrotizing enterocolitis

Erythroblastosis fetalis

Fetal demise of a twin

MISCELLLANEOUS

Severe acute graft rejection

Acute hemolytic transfusion reaction

Severe collagen-vascular disease

Kawasaki disease

Heparin-induced thrombosis

Infusion of “activated” prothrombin complex concentrates

Hyperpyrexia/encephalopathy, hemorrhagic shock syndrome

Placental abruption

Modified from Montgomery RR, Scott IP: Hemostasis: diseases of the fluid phase. In Nathan DG, Oski FA, editors: Hematology of infancy and childhood, vol 2, ed 4, Philadelphia, 1993, WB Saunders.

Clinical Manifestations

DIC accompanies a severe systemic disease process, usually with shock. Bleeding frequently first occurs from sites of venipuncture or surgical incision. The skin may show petechiae and ecchymoses. Tissue necrosis may involve many organs and can be most spectacularly seen as infarction of large areas of skin, subcutaneous tissue, or kidneys. Anemia caused by hemolysis may develop rapidly, owing to microangiopathic hemolytic anemia.

Laboratory Findings

There is no well-defined sequence of events. Certain coagulation factors (factors II, V, and VIII, and fibrinogen) and platelets may be consumed by the ongoing intravascular clotting process, with resultant prolongation of the prothrombin, partial thromboplastin, and thrombin times. Platelet counts may be profoundly depressed. The blood smear may contain fragmented and burr- and helmet-shaped red blood cells (schistocytes). In addition, because the fibrinolytic mechanism is activated, fibrinogen degradation products (FDPs, D-dimers) appear in the blood. The D-dimer is formed by fibrinolysis of a cross-linked fibrin clot. The D-dimer assay is as sensitive as the FDP test and more specific for activation of coagulation and fibrinolysis.

Treatment

The 1st 2 steps in the treatment of DIC are the most critical: (1) treat the trigger that caused DIC and (2) restore normal homeostasis by correcting the shock, acidosis, and hypoxia that usually complicate DIC. If the underlying problem can be controlled and the patient stabilized, bleeding quickly ceases, and there is improvement of the abnormal laboratory findings. Blood components are used for replacement therapy in patients with hemorrhage and may consist of platelet infusions (for thrombocytopenia), cryoprecipitate (for hypofibrinogenemia), and/or fresh frozen plasma (for replacement of other coagulation factors and natural inhibitors).

In DIC associated with sepsis, a controlled trial of drotrecogin alpha (activated protein C concentrate [APC]) in adults with sepsis showed a statistically significant survival advantage in those treated with APC. Clinical trials using protein C concentrate in purpura fulminans and APC in children with sepsis syndrome have not shown a statistically significant improvement.

The role of heparin in DIC is limited to patients who have vascular thrombosis in association with DIC or who require prophylaxis because they are at high risk for venous thromboembolism. Such individuals should be treated as outlined in Chapter 473.1, with careful attention to replacement therapy to maintain an adequate platelet count and thus limit bleeding complications.

The prognosis of patients with DIC is primarily dependent on the outcome of the treatment of the primary disease and prevention of end-organ damage.

Bibliography

Franchini M, Manzato F. Update on the treatment of disseminated intravascular coagulation. Hematology. 2004;9:81-85.

Levi M. Disseminated intravascular coagulation. Crit Care Med. 2007;35:2191-2195.

Levi M, Toh CH, Thachil J, et al. Guidelines for the diagnosis and management of disseminated intravascular coagulation. British Committee for Standards in Haematology. Br J Haematol. 2009;145:24-33.

Nadel S, Goldstein B, Williams MD, et al. Drotrecogin alfa (activated) in children with severe sepsis: a multicentre phase III randomised controlled trial. Lancet. 2007;369:836-843.

Nguyen TC, Carcillo JA. Bench-to-bedside review: thrombocytopenia-associated multiple organ failure—a newly appreciated syndrome in the critically ill. Crit Care. 2006;10:235.

Pipe SW, Goldenberg NA. Acquired hemostatic defects. In: Orkin SH, Nathan DG, Ginsberg D, et al, editors. Nathan and Oski’s hematology of infancy and childhood. ed 7. Philadelphia: Saunders Elsevier; 2009:1591-1622.

Sparrow A, Willis F. Management of septic shock in childhood. Emerg Med Australas. 2004;16:125-134.

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