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Disseminated Intravascular Coagulation, Emergency Medicine

Basics

Description

- Trauma: - Crush injury - Severe burns - Severe head injury - Fat embolism

- Miscellaneous: - Snake bites - Recreational drugs

- Excessive thrombosis: - Large vessels - Microvascular thrombosis and end organ dysfunction - Cardiac, pulmonary, renal, hepatic, CNS - Thrombophlebitis - Pulmonary embolus - Nonbacterial thrombotic endocarditis - Gangrene - Ischemic infarcts of kidney, liver, CNS, bowel

- Inherited coagulation disorders: - Other acquired coagulation disorders: - Anticoagulant therapy - Drugs - Hepatic disease - Vitamin K deficiency - Massive blood loss

- Airway management and resuscitation measures: - Control bleeding - Establish IV access - Restore and maintain circulating blood volume.

- Initiate therapy of precipitating disease: - Antibiotics in sepsis - Evacuate uterus of retained products of conception - Chemotherapy in malignancy - D пїЅbridement of devitalized tissue in trauma

- Therapy of DIC is controversial and should be individualized based on: - Age - Hemodynamic status - Severity of hemorrhage - Severity of thrombosis

- Recombinant factor VIIa - Successful use reported, benefit and safety unknown.

- Washed packed cells - Albumin - Nonclotting volume expanders

- Inhibit intravascular clotting with heparin: - Use is controversial. - Consider when thrombosis predominates. - May be effective in mild to moderate DIC - Efficacy undetermined in severe DIC. Possible indications: - Purpura fulminans (gangrene of digits, extremities) - Acute promyelocytic leukemia - Dead fetus syndrome-several weeks after intrauterine fetal death - Thromboembolic complications of large vessels - Before surgery with metastatic carcinoma

- Inhibit fibrinolysis: - Block secondary compensatory fibrinolysis that accompanies DIC - Use complicated by severe thrombosis - Use only when DIC accompanied by primary fibrinolysis: - Promyelocytic leukemia - Giant hemangioma - Heat stroke - Amniotic fluid embolism - Metastatic carcinoma of prostate

  • Normal coagulation: Series of local reactions among blood vessels, platelets, and clotting factors
  • Disseminated intravascular coagulation (DIC) is systemic activation of coagulation and fibrinolysis by some other primary disease process.
  • Coagulation system activation results in systemic circulation of thrombin and plasmin.
  • Role of thrombin in DIC:Tissue factor/factor VIII(a) activate the extrinsic pathway, leads to thrombin formation.Thrombin circulates and converts fibrinogen to fibrin monomer.Fibrin monomer polymerizes into fibrin (clot) in the circulation.Clots cause microvascular and macrovascular thrombosis with resultant peripheral ischemia and end organ damage.Platelets become trapped in clot with resultant thrombocytopenia.
  • Role of plasmin in DIC:Plasmin circulates systemically converting fibrinogen into fibrin degradation products (FDPs).FDPs combine with fibrin monomers.FDP-monomer complexes interfere with normal polymerization and impair hemostasis.FDPs also interfere with platelet function.
  • Role of impaired anticoagulation in DIC.Failure of physiologic anticoagulation is necessary for DIC to occur.Antithrombin III, protein C system, and tissue factor pathway inhibitor all impaired.
  • Acute DIC-uncompensated form:Clotting factors used more rapidly than body can replace themHemorrhage predominant clinical feature, which overshadows ongoing thrombosis
  • Chronic DIC-compensated form:Body able to keep up with pace of clotting factor consumptionThrombosis predominant clinical feature

Etiology

  • Precipitated by many disease states
  • Complications of pregnancy:Retained fetusAmniotic fluid embolismPlacental abruptionAbortionEclampsiaHELLP syndrome
  • Sepsis:Gram negative (endotoxin-mediated meningococcemia)Gram positive (mucopolysaccharide-mediated)Other microorganisms (e.g., viruses, parasites)
  • Trauma:Crush injurySevere burnsSevere head injuryFat embolism
  • Malignancy:Solid tumor or metastatic diseaseHematologic malignancy (e.g., leukemia)
  • Intravascular hemolysis:Transfusion reactionsMassive transfusion
  • Organ destruction:Severe pancreatitisSevere hepatic failure
  • Vascular abnormalities:Kasabach-Merritt syndromeLarge vascular aneurysm
  • Thrombocytopenia:Thrombotic thrombocytopenic purpuraIdiopathic thrombocytopenic purpura
  • Miscellaneous:Snake bitesRecreational drugs

Diagnosis

Signs and Symptoms

  • Excessive bleeding:PetechiaePurpuraHemorrhagic bullaeWound bleedingBleeding from venipuncture/arterial linesEpistaxisHemoptysisGI bleeding
  • Excessive thrombosis:Large vesselsMicrovascular thrombosis and end organ dysfunctionCardiac, pulmonary, renal, hepatic, CNSThrombophlebitisPulmonary embolusNonbacterial thrombotic endocarditisGangreneIschemic infarcts of kidney, liver, CNS, bowel
  • Acute DIC:Hemorrhagic complications predominate.
  • Chronic DICL:Thrombotic complications predominate.

History

  • Previous history of bleeding disorder
  • Pregnancy/last menstrual period
  • History of malignancy or immunocompromised

Physical Exam

  • Neurologic:Altered MS, confusion, lethargy
  • Cardiovascular:
  • Respiratory:Tachypnea, rhonchi, rales
  • GI:Upper or lower GI bleeding, abdominal distension
  • GU:
  • Skin:Petechiae, purpura, jaundice, necrosis

Essential Workup

  • Depends on precipitating illness
  • Diagnosis generally not made in ED

Diagnosis Tests & Interpretation

Lab

  • Platelet count:Important to note rapid decrease<100,000/mm3May be normal in chronic DIC
  • Prothrombin time (PT)/partial thromboplastin time (PTT):IncreasedMay be normal in chronic DIC
  • Fibrinogen:Decreased<150 mg/dL in 70%Low sensitivity, as levels can remain normalMay be normal in chronic DIC
  • FDPs:
  • D-dimer increased
  • CBC/peripheral smear:Red cell fragmentsLow plateletsPeripheral smear confirms disease in chronic DIC
  • Electrolytes, BUN, creatinine, glucose:Elevated BUN, creatinine owing to renal insufficiency
  • ABGs:
  • ISTH scoring systemUnderlying disorder associated with DICPlatelet count>100 = 0, <100 = 1, <50 = 2Fibrin markers (D-dimer, FDP)Normal = 0, moderate increase = 1, strong increase = 2Prolonged PT<3 = 0, >3 but <6 = 1, >6 = 2FibrinogenScore >5 overt DIC, associated with increased mortality.

Imaging

  • CXR for suspected pneumonia
  • Head CT for altered mental status
  • OB US in pregnant patients

Differential Diagnosis

  • Inherited coagulation disorders:
  • Other acquired coagulation disorders:Anticoagulant therapyDrugsHepatic diseaseVitamin K deficiencyMassive blood loss
  • Platelet dysfunction:
  • Platelet dysfunction:

Treatment

Initial Stabilization/Therapy

  • Airway management and resuscitation measures:Control bleedingEstablish IV accessRestore and maintain circulating blood volume.
  • Initiate therapy of precipitating disease:Antibiotics in sepsisEvacuate uterus of retained products of conceptionChemotherapy in malignancyD пїЅbridement of devitalized tissue in trauma

Ed Treatment/Procedures

  • Therapy of DIC is controversial and should be individualized based on:AgeHemodynamic statusSeverity of hemorrhageSeverity of thrombosis
  • Involve admitting service before initiating specific DIC therapy.
  • Replace depleted blood components:Fresh frozen plasma (FFP):For prolonged PTProvides clotting factors and volume replacementDose: 2 U or 10-15 mL/kgPlatelets:If platelet count <20,000 or platelet count <50,000 with ongoing bleedingDose: 1 U/10 kg body weightCryoprecipitate:Higher fibrinogen content than whole plasmaFor severe hypofibrinogenemia (<50 mg/dL) or for active bleeding with fibrinogen <100 g/dLDose: 8 URecombinant factor VIIaSuccessful use reported, benefit and safety unknown.Washed packed cellsAlbuminNonclotting volume expanders
  • Inhibit intravascular clotting with heparin:Use is controversial.Consider when thrombosis predominates.May be effective in mild to moderate DICEfficacy undetermined in severe DIC. Possible indications:Purpura fulminans (gangrene of digits, extremities)Acute promyelocytic leukemiaDead fetus syndrome-several weeks after intrauterine fetal deathThromboembolic complications of large vesselsBefore surgery with metastatic carcinomaAdminister activated protein C (controversial):AntithrombinNo mortality benefit found in patients also receiving heparin.Lack of evidence to support use at this time.
  • Inhibit fibrinolysis:Block secondary compensatory fibrinolysis that accompanies DICUse complicated by severe thrombosisUse only when DIC accompanied by primary fibrinolysis:Promyelocytic leukemiaGiant hemangiomaHeat strokeAmniotic fluid embolismMetastatic carcinoma of prostateInitiate in extreme cases only:Profuse bleeding not responding to replacement therapyExcessive fibrinolysis present (rapid whole blood lysis/short euglobulin lysis time)E-aminocaproic acid (EACA)Tranexamic acid

Medication

Specific DIC treatment is usually not initiated in the ED. Underlying precipitating diseases should be treated initially: пїЅ

  • Heparin:Low-dose regimen: 5-10 U/kg/h IV for causes where thrombosis predominates.

Follow-Up

Disposition

Admission Criteria

Severe precipitating illness in combination with DIC requires ICU admission. пїЅ

Discharge Criteria

None пїЅ

Followup Recommendations

Follow-up involves following platelets and coagulation factors. пїЅ

Pearls and Pitfalls

  • Suspect DIC as a complicating factor in severe, life-threatening illness.
  • Establish early clinical suspicion since the sequelae of DIC can be devastating.
  • Remember to consider treating the underlying cause of DIC when the thromboembolic and bleeding complications of the process seem to be dominating the clinical picture.

Additional Reading

  • Bick пїЅRL. Disseminated intravascular coagulation current concepts of etiology, pathophysiology, diagnosis, and treatment. Hematol Oncol Clin North Am. 2003;17(1):149-176.
  • Levi пїЅM. Disseminated intravascular coagulation. Crit Care Med. 2007;35:2191-2195.
  • Levi пїЅM, Toh пїЅCH, Thachil пїЅJ, et al. Guidelines for the diagnosis and management of disseminated intravascular coagulation. British Committee for Standards in Haematology. Br J Haematol. 2009;145(1):24-33.
  • Levi пїЅM, van der Poll пїЅT. Disseminated intravascular coagulation: A review for the internist. Intern Emerg Med. 2013;8:23-32.
  • Rodgers пїЅGM. Acquired coagulation disorders. In: Greer пїЅJP, Foerster пїЅJ, Rodgers пїЅGM, et al., eds. Wintrobes Clinical Hematology. 12th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2009:1422-1455.

See Also (Topic, Algorithm, Electronic Media Element)

  • Sepsis
  • Idiopathic Thrombocytopenic Purpura
  • Thrombotic Thrombocytopenic Purpura

Codes

ICD9

286.6 Defibrination syndrome пїЅ

ICD10

D65 Disseminated intravascular coagulation пїЅ

SNOMED

  • 67406007 Disseminated intravascular coagulation (disorder)