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Disseminated Intravascular Coagulation CE Article Disseminated Intravascular Coagulation Yaron Bruchim , DVM Itamar Aroch , DVM, DECVIM-CA Joseph Saragusty , DVM The Hebrew University of Jerusalem, Israel Trevor Waner , BVSc, PhD , DECLAM Israel Institute for Biological Research, Ness Ziona, Israel ABSTRACT: Disseminated intravascular coagulation (DIC) is a serious, life-threatening condition in humans and animals. A secondary complication in a variety of disorders, it is a complex syndrome in which excessive intravascular coagulation leads to microthromboses in and consequential failure of multiple organs with concurrent paradoxical bleeding due to inactivation and excessive consumption of platelets and clotting factors. This article discusses the pathophysiology, diagnosis, and treatment of DIC in dogs and cats. Novel treatments and laboratory tests, some of which are still being experimentally evaluated, are also discussed. isseminated intravascular coagulation so that coagulation and clot formation occur only (DIC) is a serious , life -threatening compli - on demand. In DIC, this balance is disrupted, Dcation in humans and animals, previously leading to concurrent excessive clot formation called consumptive coagulopathy or defibrination and bleeding (Figure 1). Thus, DIC may be con - syndrome .1 In this complex syndrome , excessive sidered as an uncontainable burst of thrombin intravascular coagulation leads to diffuse generation and activation resulting in systemic microthromboses and subsequent failure of multi - fibrin formation , plasmin activation, suppression ple organs . Inactivation and excessive consumption of the physiologic anticoagulation mechanisms , of platelets and clotting factors cause concurrent and delayed fibrin removal as a consequence of paradoxical bleeding. DIC is not a primary disease impaired fibrinolysis. 5,6 During this excessive but rather a secondary complication of several intravascular coagulation phase, platelets and disorders 1–3 (Box 1) . Moreover, it constitutes a coagulation factors are consumed, resulting in dynamic phenomenon during which the patient ’s thrombocytopenia, thrombocytopathy , and deple - status and laboratory coagulation test results may tion and inactivation of coagulation factors .6–8 fluctuate markedly, rapidly, and repeatedly. It is re- DIC is an acquired syndrome that occurs as a latively common in dogs and uncommon in cats .4 result of a primary disorder. Three factors, com - monly termed Virchow’s triad , predispose PATHOPHYSIOLOGY patients to thrombosis: stasis, hypercoagulabil ity, In healthy animals, the normal mechanisms of and blood vessel wall injury .9 Therefore, any dis - clot formation and fibrinolysis are well balanced ease process that results in capillary stasis, loss of October 2008 •Vol. 30, No. 10 E1 COMPENDIUM E2 CE Disseminated Intravascular Coagulation Box 1. Clinical Conditions Potentially Associated with DIC Systemic bacterial infections/sepsis Immunologic disorders • Gram -negative bacteria (endotoxin) • Immune -mediated hemolytic anemia • Gram -positive bacteria (bacterial coat • Hemolytic transfusion reaction mucopolysaccharide, enzymes) • Transplant rejection Viral diseases Vascular disorders • Canine parvovirus • Feline panleukopenia • Aortic aneurysm • Hemangioma • Infectious canine hepatitis • FIP • Vasculitis Canine parasitic and rickettsial infections Massive tissue injury • Babesiosis ( Babesia canis rosi ) • Heatstroke and hyperthermia • Monocytic ehrlichiosis ( Ehrlichia canis ) and Rocky • Gastric dilatation –volvulus (canine ) Mountain spotted fever ( Rickettsia rickettsii ) • Burns • Leishmaniasis ( Leishmania infantum ) • Head trauma • Spirocercosis ( Spirocerca lupi ) • Fat embolism • Heartworm disease ( Dirofilaria immitis ) • Surgery (especially extensive and orthopedic ) • Caval syndrome ( Angiostrongylus vasorum and • Severe mechanical trauma Dirofilaria immitis ) Reaction to toxins Neoplasia • Snakebite • Solid tumors (e.g. , mammary tumors) • Lymphoid leukemia Miscellaneous • Myeloproliferative disorders • Pancreatitis • Hepatic failure • Lymphoma • Polycythemia • Hemangiosarcoma (canine) • Pulmonary adenocarcinoma vascular integrity , or hypercoagulability (e.g., the presence lants , and delayed fibrin removal as a consequence of of inappropriate matter in the bloodstream, tissue necrosis ) inadequate fibrinolysis .18,19 In addition, inflammation can lead to disruption of the balance between hemostasis tends to lead to an increase in fibrinogen levels, unless and fibrinolysis and subsequently induce DIC .5,8 Depend - fibrinogen consumption is concurrent .20,21 In sepsis or ing on the activation rate of the hemostatic system, DIC inflammation, the propagation of the coagulation cas - may present on the one extreme as a peracute , life -threat - cade is also promoted by a reduction in the physiologic ening clinical event and on the other as a chronic condition anticoagulation activity. With extensive clot formation, with no overt hemorrhage or thrombosis .5,8,10,11 antithrombin (AT) is consumed by binding in a one-to- Many common systemic diseases associated with one fashion with each clotting factor. Moreover, the inflammation have been reported to initiate DIC in presence of endotoxin or inflammatory mediators dogs and cats 12–17 (Box 1) . In a recent study 13 that reduces the endothelial expression of glycosaminogly - included 164 dogs with solid tumors, 12.2% of the dogs cans, such as heparan sulfate, that normally augment the presented with DIC, and dogs with hemangiosarcoma, activity of AT, leading to reduced AT activity and mammary gland carcinoma , or pulmonary adenocarci - endothelial antithrombotic function 22–26 (Figure 2 ). noma had a significantly higher incidence of DIC . In another study 12 of 54 dogs with heatstroke, 50% pre - Generation of Thrombin sented with DIC . The systemic generation of thrombin in animal mod - Regardless of whether the intrinsic or extrinsic activa - els of DIC is mediated exclusively by the extrinsic path - tion pathway is initiated, once triggered, DIC follows way , involving tissue factor and activated factor VII the same course . Only recently has the importance of (VIIa ). 18 Levi and colleagues 27 showed that inhibition of the inflammatory mechanism in the up-regulation of tissue factor or factor VIIa totally suppressed the endo - procoagulant factors been recognized. This up -regula - toxin-induced generation of thrombin, whereas interfer - tion results in increased thrombin generation, systemic ence in the intrinsic pathway of coagulation did not fibrin formation, down -regulation of natural anticoagu - affect the activation of coagulation . COMPENDIUM Vol. 30, No. 10 • October 2008 Disseminated Intravascular Coagulation CE E3 Precipitating event (Box 1) Tissue factor/extrinsic pathway activation Coagulation cascade Excess thrombin Conversion of plasminogen to plasmin Microvascular Macrovascular clotting clotting Thrombocytopenia Consumption of Fibrinolysis with clotting factors excess FDPs Excess clotting Excess bleeding Ischemia Shock Impaired organ perfusion Hypotension End-organ damage Increased vascular permeability Figure 1. Mechanisms leading to the development of DIC. FDP = fibrin degradation products . (Adapted from Gobel BH. Disseminated intravascular coagulation in cancer: providing quality care. Topics Adv Pract Nurs J [online] 2002;2(4). Accessed October 2008 at www.medscape.com) The conditions that lead to DIC are the same as those propagation of the coagulation cascade to take place. To associated with systemic inflammatory response syndrome express the optimal procoagulant lipid surface, potent cell and are characterized by activation of cytokine production. agonists, such as a combination of collagen and thrombin, The principal mediators are interleukins 1 and 6 and are required .30 In addition, increased interleukin-1 levels tumor necrosis factor (TNF), which are released from the have been shown to increase platelet reactivity and throm - monocyte –macrophage system .8,18,28,29 These cytokines bogenic potential .31 stimulate macrophages to express several procoagulant moieties (mainly tissue factor ) on their outer surface .21,29 Impairment of Fibrinolysis and the Role of The reactions involved in coagulation initiation and Fibrin Degradation Products amplification require a membrane surface that contains Studies in animal models of DIC indicate that the fib - negatively charged phospholipids. Normally, these nega - rinolytic system is largely suppressed when coagulation is tively charged phospholipids are not expressed on cell sur - maximally activated. This inhibition is caused by up -reg - faces in sufficient concentrations for initiation and ulation and a sustained increase in the plasma level of October 2008 •Vol. 30, No. 10 COMPENDIUM E4 CE Disseminated Intravascular Coagulation plasminogen activator inhibitor, the principal inhibitor of activated protein C (APC) , and tissue factor pathway the fibrinolytic system, as well as depression of tissue inhibitor (TFPI), which are all consumed during this plasminogen activator production .8,27,32 Fibrin deposition phase .18,21,29 AT, when combined with heparin, is a potent forms multiple microthrombi within small blood vessels, inhibitor of thrombin and factor Xa and inactivates fac - leading to tissue ischemia and necrosis in vital organs .7,33 tors XIIa, XIa, IXa, and VIIa ; plasma kallikrein ; and plas - Although the fibrinolytic system is largely activated min .44–46 Thus, under normal conditions , the activity of through the actions
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