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Disseminated Intravascular Coagulation

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Disseminated Intravascular Coagulation Disseminated Intravascular CHAPTER Coagulation 29 Velu Nair Introduction The syndrome can be considered to involve a 2 Disseminated intravascular coagulation (DIC) is phase phenomenon: a hypercoagulable phase that an acquired hypercoagulable state, induced by the leads into a hypocoagulable phase manifesting in progressive generation of thrombin in circulation. It bleeding. Most physicians encounter patients in the is a pathophysiologic term describing a continuum hypocoagulable state with bleeding manifestations in of events that occur in the coagulation pathway as critical illnesses. The thrombotic complications are a complication of many different serious and life- more frequently seen in patients with malignancies threatening disease states. The International Society who run a chronic course. In acute promyelocytic on Thrombosis and Hemostasis has suggested leukemia the patient usually presents with bleeding the following definition for DIC: “An acquired tendency secondary to DIC triggered by the syndrome, characterized by the intravascular granules released from the promyelocytes. activation of coagulation with loss of localization DIC is initiated by the activation of the arising from different causes. It can originate clotting cascade, mainly the tissue factor (TF) from and cause damage to the microvasculature, pathway. Diagnosis of DIC can be difficult, which if sufficiently severe, can produce organ depending upon the phase in which a diagnosis is dysfunction.1 DIC occurs in acute and chronic attempted, since many laboratory parameters can forms. In its acute (overt) form it is a hemorrhagic be normal in the hypercoagulable phase except disorder, characterized by multiple ecchymoses, for a falling platelet count. The diagnosis of the mucosal bleeding and consumption of platelets hypocoagulable phase is easier, as the patient and clotting factors. Chronic (nonovert) DIC, manifests clinical bleeding with abnormalities in on the other hand, is more subtle and involves global coagulation parameters. venous thromboembolism (VTE) accompanied by DIC is seen in association with a number of well- evidence of activation of the coagulation system defined clinical situations, including sepsis, major and is associated with conditions like malignancies trauma, and abruptio placenta with laboratory and intrauterine death of fetus. With chronic DIC, evidence of the following1 coagulation factors may be normal, increased, or moderately decreased, as may the platelet • Activation of procoagulant pathway counts.2 • Activation of fibrinolytic pathway Disseminated Intravascular Coagulation 231 • Inhibitor (natural anticoagulants) The mortality rates in different diseases complicated consumption by DIC is as under: • Evidence of end-organ damage or failure • Septic abortion with shock due to clostridial Though numerous laboratory tests are available infection associated with severe DIC has a to monitor DIC, most patients are managed mortality rate of 50%. successfully using only routine screening tests, • In major trauma, the presence of DIC platelet counts and assays for fibrinogen and approximately doubles the mortality rate. D-dimer. Treatment of DIC should focus on • Idiopathic purpura fulminans associated with reversing the underlying disorder initiating the DIC has a mortality rate of 18%. coagulopathy. Supportive care with appropriate Sex: Incidence is equal in males and females. blood components is paramount to minimise the mortality and morbidity. By and large, the outcome Age: No age predilection is known. of DIC is dictated by the severity of the underlying disorders leading to DIC and how successfully Pathogenesis they can be managed. Novel treatments are being The pathogenetic pathways of DIC have been largely investigated for treating DIC, many of which target clarified by recent studies in animal models and humans. the excessive TF activity that characterizes DIC. DIC occurs when monocytes and endothelial cells are activated by toxic substances elaborated in the course Frequency of certain diseases. These cells generate TF, which • DIC may occur in 30-50% of patients with sepsis in turn activates the coagulation cascade generating with equal frequency in gram negative and thrombin. Thrombin leads to systemic formation positive bacterial infections. In patients with of fibrin.3 Simultaneously, there is consumption of severe trauma who have a systemic inflammatory naturally occurring anticoagulants and a delayed response syndrome, DIC can occur in 50-70% removal of fibrin due to impairment in the fibrinolytic patients, particularly in patients with neuro- pathways. Several pro-inflammatory cytokines play a trauma. In obstetrical patients with abruptro pivotal role in the causation of systemic inflammatory placentae, septic abortions and amniotic fluid response. The principal cytokines involved appears to embolism, DIC occurs in more than 50% be interleukin -6, interleukin -1β and tumor necrosis patients. Cancer patients with metastatic tumors factor-α which are active in both polytrauma and 4 have DIC in approximately 10-15% patients and sepsis. it occurs in 15% cases with acute leukemia with Thrombin is generated predominantly by the highest frequency in APML.1 extrinsic pathway (involving tissue factor and • Incidence of DIC varies with the energy with activated factor VII) the inhibition of which totally which the diagnosis is pursued. High index suppressed endotoxin induced thrombin generation of clinical suspicion is warranted in a clinical in animal models. However, interference with the setting for DIC. The incidence is 1:1000 intrinsic pathway did not in anyway affect activation 4 admissions in a general hospital. Most of these of coagulation. data is from the West and there is a need to There is a decrease in the levels of all major generate epidemiological data from India. physiological anti-coagulants like anti-thrombin III, protein C and tissue factor pathway inhibitor Morbidity / Mortality (TFPI). Anti-thrombin III which is the most potent Morbidity and mortality depend on both, the thrombin inhibitor, is markedly reduced due to underlying disease and the severity of coagulopathy. consumption, impaired synthesis and degradation 232 Medicine Update 2008 Vol. 18 Table 1 : Conditions underlying DIC syndrome cutaneous bleeds should always raise the possibility Infections of DIC in the ICU setting. Look for symptoms Acute DIC: Bacteria and their toxins, fungi, viruses, and signs of thrombosis in large vessels, such as rickettsiae Chronic DIC: Chronic infection. e.g., tuberculosis, DVT, and of microvascular thrombosis, such as abscesses, osteomyelitis renal failure. Bleeding from at least 3 unrelated Obstetrical complications sites is particularly suggestive of DIC. Patient can Acute DIC: Abruptio placentae, abortions (especially therapeutic and septic abortions), amniotic fluid present with varied symptoms due to affection of embolism, hemorrhagic shock any organ in the body. The common presentation Chronic DIC: Dead fetus syndrome include. Trauma Acute DIC: Polytrauma; neurotrauma • Skin ecchymosis, bleeding from I/V sites, Venoms endotracheal tubes and urinary catheters Acute DIC: Snake bites and rarely spider bites • Gingival bleeding, epistaxis Malignancy Acute DIC: Acute promyelocytic leukemia, acute • Cough, dyspnea myelomonocytic or monocytic leukemia, disseminated prostatic carcinoma • Confusion, disorientation Chronic DIC: Gastrointestinal, lung and breast • Fever malignancy Vascular disease Acute and Chronic DIC Acute DIC: Brain infarction or hemorrhage Chronic DIC: Aortic aneurysm, giant hemangioma Acute DIC is the most commonly seen form in clinical Noninfectious inflammatory diseases practice and presents with bleeding manifestations Inflammatory bowel disease: Crohn’s disease and similar from various sites. It is a hematological emergency disorders and is rapidly progressive because of explosive Others generation of thrombin. Prothrombin time (PT) Acute DIC: Heparin-induced thrombocytopenia with thrombosis (HITT); purpura fulminans in newborns and activated partial thromboplastin time (APTT) (homozygous protein C deficiency); transfusion of ABO are prolonged along with hypofibrinogenemia and incompatible red cells. thrombocytopenia. FDP and D-Dimer levels are by elastase released from activated neutrophils. increased. The decrease in total protein-C levels is caused Chronic DIC is less explosive as there is time by consumption and impaired synthesis. The fibrinolytic system is suppressed at the time of for compensatory responses to take place. Usually maximal activation of coagulation. This inhibition presents with features of hyper-coagulable state is caused by a sustained increase in the levels of with or without VTE. Platelet levels are low plasminogen activator inhibitor type -1 (PAI-1). along with raised levels of FDP and D-Dimer. PT Though secondary fibrionolysis occurs in response and APTT may be normal or mildly prolonged. to fibrin formation, its activity is too low to APTT may be shortened in the hyper-coagulable counteract systemic deposition of fibrin. phase. Clinical Features Conditions Associated with DIC In addition to the symptoms related to the The conditions that regularly give rise to the underlying disease, ascertain history of blood loss DIC syndrome are outlined in Table 1. A high such as gastrointestinal bleeding and hypovolemia. index of clinical suspicion in a given setting helps Fresh bleeding from the percutaneous intravenous anticipate onset of DIC with timely intervention
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