Potential – MS, MG, stroke, aneurysm, SAH, lymphocytic meningitis, Wernicke encephalopathy
CODES
ICD9
- 368.2 Diplopia
- 368.15 Other visual distortions and entoptic phenomena
DISSEMINATED INTRAVASCULAR COAGULATION
Steven H. Bowman
•
Ernesto J. Romo
BASICS
DESCRIPTION
- 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 them
- Hemorrhage
predominant clinical feature, which overshadows ongoing thrombosis
- Chronic DIC—compensated form:
- Body able to keep up with pace of clotting factor consumption
- Thrombosis
predominant clinical feature
ETIOLOGY
- Precipitated by many disease states
- Complications of pregnancy:
- Retained fetus
- Amniotic fluid embolism
- Placental abruption
- Abortion
- Eclampsia
- HELLP syndrome
- Sepsis:
- Gram negative (endotoxin-mediated meningococcemia)
- Gram positive (mucopolysaccharide-mediated)
- Other microorganisms (e.g., viruses, parasites)
- Trauma:
- Crush injury
- Severe burns
- Severe head injury
- Fat embolism
- Malignancy:
- Solid tumor or metastatic disease
- Hematologic malignancy (e.g., leukemia)
- Intravascular hemolysis:
- Transfusion reactions
- Massive transfusion
- Organ destruction:
- Severe pancreatitis
- Severe hepatic failure
- Vascular abnormalities:
- Kasabach–Merritt syndrome
- Large vascular aneurysm
- Thrombocytopenia:
- Thrombotic thrombocytopenic purpura
- Idiopathic thrombocytopenic purpura
- Miscellaneous:
- Snake bites
- Recreational drugs
DIAGNOSIS
SIGNS AND SYMPTOMS
- Excessive bleeding:
- Petechiae
- Purpura
- Hemorrhagic bullae
- Wound bleeding
- Bleeding from venipuncture/arterial lines
- Epistaxis
- Hemoptysis
- GI bleeding
- 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
- 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 & NTERPRETATION
Lab
- Platelet count:
- Important to note rapid decrease
- <100,000/mm
3
- May be normal in chronic DIC
- Prothrombin time (PT)/partial thromboplastin time (PTT):
- Increased
- May be normal in chronic DIC
- Fibrinogen:
- Decreased
- <150 mg/dL in 70%
- Low sensitivity, as levels can remain normal
- May be normal in chronic DIC
- FDPs:
- D-dimer increased
- CBC/peripheral smear:
- Red cell fragments
- Low platelets
- Peripheral smear confirms disease in chronic DIC
- Electrolytes, BUN, creatinine, glucose:
- Elevated BUN, creatinine owing to renal insufficiency
- ABGs:
- ISTH scoring system
- Underlying disorder associated with DIC
- Platelet count
- >100 = 0, <100 = 1, <50 = 2
- Fibrin markers (D-dimer, FDP)
- Normal = 0, moderate increase = 1, strong increase = 2
- Prolonged PT
- <3 = 0, >3 but <6 = 1, >6 = 2
- Fibrinogen
- Score >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 therapy
- Drugs
- Hepatic disease
- Vitamin K deficiency
- Massive blood loss
- Platelet dysfunction:
- Platelet dysfunction:
TREATMENT
INITIAL STABILIZATION/THERAPY
- 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
- Débridement of devitalized tissue in trauma
ED TREATMENT/PROCEDURES
- Therapy of DIC is controversial and should be individualized based on:
- Age
- Hemodynamic status
- Severity of hemorrhage
- Severity of thrombosis
- Involve admitting service before initiating specific DIC therapy.
- Replace depleted blood components:
- Fresh frozen plasma (FFP):
- For prolonged PT
- Provides clotting factors and volume replacement
- Dose: 2 U or 10–15 mL/kg
- Platelets:
- If platelet count <20,000 or platelet count <50,000 with ongoing bleeding
- Dose: 1 U/10 kg body weight
- Cryoprecipitate:
- Higher fibrinogen content than whole plasma
- For severe hypofibrinogenemia (<50 mg/dL) or for active bleeding with fibrinogen <100 g/dL
- Dose: 8 U
- 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
- Administer activated protein C (controversial):
- Antithrombin
- No 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 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
- Initiate in extreme cases only:
- Profuse bleeding not responding to replacement therapy
- Excessive fibrinolysis present (rapid whole blood lysis/short euglobulin lysis time)
- E
-
aminocaproic acid (EACA)
- Tranexamic acid