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Coagulation Disorders: Causes and Types

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14 Questions

What is the result of a deficiency of Protein C or S?

Excessive clotting

Which of the following is a characteristic of a mutated Factor V?

It cannot be inhibited by Protein S or C

What is the pattern of inheritance of a deficiency of Factor VIII or IX?

X-Linked

What is the result of a deficiency of Antithrombin?

Excessive clotting

What is the diagnostic test result for a deficiency of Factor VIII or IX?

Serum levels of factors: Decreased Factor VIII or IX

What is the presentation of Deep Tissue Bleeding?

Widespread clotting, targeting organ dysfunction

What is the primary mechanism of action in ITP that leads to thrombocytopenia?

Production of autoantibodies against platelet receptors

Which of the following statements is true about Von Willebrand factor?

It helps to prolong the life of factor VIII

What is the characteristic of bleeding in Hemophilia A?

Deep tissue bleeding with prolonged PTT

What is the primary cause of hemolytic anemia in DIC?

Mechanical destruction of RBCs by clots

Which of the following is a characteristic of ITP?

Thrombocytopenia with normal PT/PTT

What is the role of endothelium in DIC?

It releases tissue factor and vWF

Which of the following is NOT a characteristic of Hemophilia A?

Thrombocytopenia

What is the primary mechanism of action in DIC?

Release of proinflammatory cytokines

Study Notes

Platelet Disorders

  • Immune Thrombocytopenic Purpura (ITP): Weak platelet plug leading to superficial bleeding due to autoimmune process, resulting in increased destruction of platelets.
  • Mechanism of action: Autoantibodies against platelet receptors, leading to platelet destruction by macrophages in the spleen, causing thrombocytopenia.

Von Willebrand Factor Disorder

  • Weak platelet plug leading to superficial bleeding due to impaired platelet function, caused by a deficiency of Von Willebrand Factor.
  • Mechanism of action: Low amount of Von Willebrand Factor, impairing platelet adhesion to collagen, leading to superficial bleeding with normal platelet count.
  • Additional effect: Decreased factor VIII, causing prolonged PTT.

Coagulation Disorders

  • Hemophilia: Insufficient production of fibrin mesh, leading to deep tissue bleeding.
  • Mechanism of action: Deficiency of factor VIII (Hemophilia A) or factor IX (Hemophilia B), leading to reduced fibrin production and deep tissue bleeding.

Mixed Disorders

  • Disseminated Intravascular Coagulation (DIC): Widespread activation of platelets and coagulation factors, leading to excessive clotting and bleeding.
  • Mechanism of action: Proinflammatory cytokines damage endothelium, releasing tissue factor and Von Willebrand Factor, leading to widespread clot formation, organ ischemia, and hemolytic anemia.
  • Later stages: Consumption of platelets and fibrin leads to bleeding.

Inherited Thrombophilia

  • Deficiency of Protein C or S, leading to unregulated clotting.
  • Deficiency of Antithrombin, leading to unregulated clotting.
  • Mutated Factor V, leading to resistance to Protein S or C, causing excessive clotting.

Patterns of Inheritance

  • Autosomal Dominant: Inherited thrombophilia affects both men and women, with a single inherited gene.
  • X-Linked Disorder: Hemophilia affects mostly males, in all ethnic groups, with females as carriers.

This quiz covers different types of coagulation disorders, including platelet disorders, coagulation factor disorders, and mixed disorders. Learn about the causes and symptoms of each type.

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