Disseminated Intravascular Coagulation
Disseminated Intravascular Coagulation: Excerpt from The 5-Minute Pediatric Consult
Char Witmer, MD
Don E. Eslin, MD (4th Edition)
Disseminated Intravascular Coagulation - BASICS
Disseminated Intravascular Coagulation - description
A systemic thrombohemorrhagic disorder that is the result of varied conditions with procoagulant activation, fibrinolytic activation, inhibitor consumption, and biochemical evidence of end-organ damage or failure.
Disseminated Intravascular Coagulation - epidemiology
- Most commonly secondary to infections
- Snakebite may be most common cause worldwide.
Disseminated Intravascular Coagulation - incidence
Exact incidence not known.
Disseminated Intravascular Coagulation - pathophysiology
- Not a disorder in itself; occurs as a result of various initiating events
- Characterized by microvascular hemorrhage and thrombosis
- May be acute (e.g., meningococcemia) or chronic (e.g., malignancy/leukemia)
- There is a systemic formation of fibrin as a result of increased thrombin generation, suppression of anticoagulant pathways, impaired fibrinolysis, and activation of inflammatory pathways.
- Thrombin formation is primarily through the extrinsic pathway, factor VIIa. This pathway is activated via tissue factor expression from damaged endothelial cells.
- Anticoagulant pathways are diminished because of a decrease in the plasma levels of antithrombin and the protein C system through impaired production and increased destruction.
- The increase in fibrinolytic activity is likely secondary to the release of plasminogen activators from damaged endothelial cells.
- The inflammatory cascade is activated via the activated coagulation proteins.
Disseminated Intravascular Coagulation - etiology
Most common causes are sepsis (particularly Gram-negative), hypotensive shock, and trauma (particularly head trauma).
- Infections:
- Bacterial Gram-negative and -positive sepsis (e.g., group B streptococcus)
- Meningococcemia
- Malaria
- Fungal: Aspergillus
- Rickettsial: Rocky Mountain spotted fever
- Viral
- Tissue injury
- Massive head trauma
- Multiple fractures with fat emboli
- Crush injuries
- Profound shock or asphyxia
- Hypothermia or hyperthermia
- Massive burns
- Malignancies:
- Acute promyelocytic leukemia
- Acute monoblastic or monocytic leukemia
- Metastatic carcinomas or other widespread malignancies (e.g., neuroblastoma)
- Obstetric:
- Retained intrauterine fetal death
- Amniotic fluid embolism
- Abruptio placentae
- Placenta previa
- GI:
- Fulminant hepatitis/hepatic failure
- Severe pancreatitis
- Reye syndrome
- Severe inflammatory bowel disease
- Neonatal:
- Necrotizing enterocolitis
- Congenital viral infections
- Group B streptococcus infection
- Erythroblastosis fetalis
- Small for gestational age
- Miscellaneous:
- Acute hemolytic transfusion reaction
- Kasabach-Merritt syndrome
- Snake bite
- Severe thrombotic thrombocytopenic purpura
- Hemolytic uremic syndrome
- Homozygous protein C deficiency (purpura fulminans)
- Severe graft rejection
- Severe collagen vascular disease
- Heparin-induced thrombosis
- Infusion of activated prothrombin complex concentrate
- Ventriculoperitoneal shunt
- Kawasaki disease
- Recreational drugs
- Hemophagocytic lymphohistiocytosis (HLH)
Disseminated Intravascular Coagulation - DIAGNOSIS
Disseminated Intravascular Coagulation - signs & symptoms
Disseminated Intravascular Coagulation - history
- Presence of one of the underlying conditions (see “Etiology”)
- Abrupt onset of bleeding
- Prolonged bleeding from venipuncture sites
- Bleeding from multiple sites, especially venipunctures, cutdown sites, mucous membranes, skin, GI tract, and genitourinary tract
- Pulmonary or intracranial hemorrhage
- Major organ dysfunction: Pulmonary, renal, hepatic
Disseminated Intravascular Coagulation - physical exam
- Signs of underlying disease
- Generally, a very toxic-appearing patient
- Ecchymosis and petechiae
- Bleeding from previously intact venipuncture sites
- Skin infarctions (purpura fulminans) secondary to thrombosis of dermal vessels
- Pulmonary hemorrhage, gastrointestinal bleeding, bleeding from surgical wounds, hematuria
- Intraperitoneal and pleural hemorrhages
Disseminated Intravascular Coagulation - tests
- There is no single test that can reliably diagnose disseminated intravascular coagulation (DIC).
- The following lab abnormalities can be seen:
- Prolonged PT in 50–75% of patients with DIC
- Prolonged aPTT in 50–60% of patients with DIC
- Elevated fibrin degradation products in 85–100% of patients with DIC
- Elevated d-dimer in 93–100% of patients with DIC (most reliable test for DIC)
- Thrombocytopenia: Range 20–100 × 109/L
- Additional findings include decreased coagulation factors, fibrinogen, antithrombin, and protein C/S
Disseminated Intravascular Coagulation - lab
The following should be followed closely because results change rapidly:
- CBC: Decreased platelet count is often the earliest abnormality.
- Peripheral smear: Schistocytes, microspherocytes (50% of cases)
- PT, aPTT, and thrombin times: Prolonged
- Fibrinogen: Decreased
- Fibrin degradation products or fibrin split products: Increased
- Soluble fibrin monomer complexes (d-dimers): Increased
- Antithrombin or protein C levels: Decreased
- Factors VIII and V: Decreased; factor VIII is normal in coagulopathy of liver disease
Disseminated Intravascular Coagulation - differencial diagnosis
- Prolonged bleeding from venipuncture sites
- Coagulopathy of liver disease
- Vitamin K deficiency
- Pathologic fibrinolysis
- Microangiopathic disease, e.g., thrombotic thrombocytopenic purpura
- Effects of cardiopulmonary bypass
Disseminated Intravascular Coagulation - TREATMENT
Disseminated Intravascular Coagulation - general measures
- The most important therapy for DIC is to treat the underlying disorder.
- Replacement therapy:
- Cryoprecipitate, platelets, and fresh frozen plasma to control bleeding
- Fresh frozen plasma also replaces anticoagulants–antithrombin, protein C and S.
- The role of heparin for DIC is controversial. It has been used in chronic DIC, arterial thromboses, or large-vessel venous thromboses.
- Antithrombin and protein C concentrates at supraphysiologic dosing have been studied with mixed results. At this time they are not considered standard of care.
- Off label use of recombinant activated factor VII has been reported for patients with severe bleeding that is refractory to replacement therapy. There are concerns about the prothrombotic potential of this medication.
- Antifibrinolytic agents (aminocaproic acid) have been used for patients with intense fibrinolysis (e.g., Kasabach Merritt or acute promyelocytic leukemia). There are concerns about the prothrombotic potential of this medication.
- Supportive care: Manage other organ system failure.
Disseminated Intravascular Coagulation - FOLLOW UP
Disseminated Intravascular Coagulation - prognosis
- Poor unless underlying disease is treated
- The intensity and duration of DIC depend on the degree of activation of the coagulation system, liver function, blood flow, and ability to reverse underlying etiology that has led to DIC.
Disseminated Intravascular Coagulation - complications
- Hemorrhage:
- Thrombosis
- Multiorgan system failure
Disseminated Intravascular Coagulation - bibliography
- Bick RL. Disseminated intravascular coagulation current concepts of etiology, pathophysiology, diagnosis, and treatment. Hematol Oncol Clin N Am. 2003;17:149–176.
- Franchini M, Lippi G, Manzato F. Recent acquisitions in the pathophysiology, diagnosis, and treatment of disseminated intravascular coagulation. Thromb J. 2006;4:4.
- Levi M. Disseminated intravascular coagulation: What’s new? Crit Care Clin. 2005;21:449–467.
- Levi M, deJonge E, van der Poll T, et al. Novel approaches to the management of disseminated intravascular coagulopathy. Crit Care Med. 2000;28(9 suppl):S20–S24.
Monagle P, Andrew M. Acquired disorders of hemostasis. In: Nathan DG, Orkin SH, Ginsburg D, et al., eds. Nathan and Oski’s Hematology of Infancy and Childhood. 6th ed. Philadelphia: WB Saunders; 2003:1597–1630.
Disseminated Intravascular Coagulation - CODES
Disseminated Intravascular Coagulation - icd9
286.6 DIC (diffuse or disseminated intravascular coagulopathy)
Book Source Details
- Book Title: The 5-Minute Pediatric Consult
- Author(s): M. William Schwartz MD; et al.
- Year of Publication: 2008
- Copyright Details: The 5-Minute Pediatric Consult, Copyright © 2008 Lippincott Williams & Wilkins.
More About DIC
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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
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More About This Book:
Title: The 5-Minute Pediatric Consult
Authors: M. William Schwartz MD; et al.
Publisher: Lippincott Williams & Wilkins
Copyright: 2008
ISBN: 0-7817-7577-9
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