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Questions and Answers
What is the consequence of the release of thromboplastins from damaged liver cells in liver disease?
What is the consequence of the release of thromboplastins from damaged liver cells in liver disease?
What is the main clinical presentation of Disseminated Intravascular Coagulation (DIC)?
What is the main clinical presentation of Disseminated Intravascular Coagulation (DIC)?
What is the cause of thrombocytopenia in liver disease?
What is the cause of thrombocytopenia in liver disease?
What is the result of impaired removal of activated clotting factors and increased fibrinolytic activity in liver disease?
What is the result of impaired removal of activated clotting factors and increased fibrinolytic activity in liver disease?
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What is the result of circulating antibodies to coagulation factors in liver disease?
What is the result of circulating antibodies to coagulation factors in liver disease?
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What is the consequence of deficient utilization of vitamin K in liver disease?
What is the consequence of deficient utilization of vitamin K in liver disease?
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What is the cause of dysfibrinogenaemia in liver disease?
What is the cause of dysfibrinogenaemia in liver disease?
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What is the consequence of coagulation factor deficiencies in liver disease?
What is the consequence of coagulation factor deficiencies in liver disease?
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What is the consequence of widespread endothelial damage in liver disease?
What is the consequence of widespread endothelial damage in liver disease?
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What percentage of patients with Disseminated Intravascular Coagulation (DIC) manifest thrombotic lesions?
What percentage of patients with Disseminated Intravascular Coagulation (DIC) manifest thrombotic lesions?
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Study Notes
DIC (Disseminated Intravascular Coagulation)
- Considered an acquired bleeding disorder, not a disease entity
- Abnormal acceleration of the coagulation cascade results in thrombosis and simultaneous hemorrhage
- Paradoxical clinical presentation: clotting and hemorrhage
- Results in depletion of clotting factors
Acquired Coagulation Disorders
- Coagulation deficiency caused by antibodies (e.g., factor VIII autoantibodies)
- Occurs rarely, with an incidence of approximately 1 per million per year
- Treatment consists of immunosuppression and factor replacement
Massive Transfusion Syndrome
- Defined as transfusion of more than 1.5 times the patient's blood volume in 24 hours
- Results in acquired coagulopathy due to dilution of plasma and platelets and excess anticoagulant
- Prevention: administer 1 unit FFP and calcium chloride for every 4-6 units of PRBCs
Disorders of Platelet Function
- Hyperglobulinaemia: associated with multiple myeloma or Waldenström's disease, causing interference with platelet adherence, release, and aggregation
- Myeloproliferative and myelodysplastic disorders: intrinsic abnormalities of platelet function
- Uraemia: associated with various abnormalities of platelet function
Hereditary Coagulation Disorders
- Hereditary deficiencies of each of the coagulation factors have been described
- Haemophilia A (factor VIII deficiency), haemophilia B (factor IX deficiency), and von Willebrand disease are the most frequent
- Other deficiencies (e.g., fibrinogen, prothrombin, factors V, VII, X, XI, XIII) are rare
- Inheritance is autosomal recessive, except for factor XI deficiency
- Recombinant factor VIIa is available for therapy
Factor XI Deficiency
- Seen mainly in Ashkenazi Jews, occurring in either sex
- Bleeding risk shows incomplete correlation to the severity of the deficiency
- Bleeding occurs after trauma, such as surgery
- Treatment is with fibrinolytic inhibitor, factor XI concentrate, or fresh frozen plasma (FFP)
Factor XIII Deficiency
- Produces a severe bleeding tendency, characteristically with umbilical stump bleeding
- Plasma concentrates and recombinant preparations of factor XIII are available
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Description
This quiz covers the characteristics of acquired coagulation disorders, including their paradoxical clinical presentation and the underlying mechanisms of blood clotting and hemorrhage.
