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  • Image not available. Hemophilia is an inherited bleeding disorder resulting from a congenital deficiency in factor VIII or IX.
  • Image not available. The goal of therapy for hemophilia is to prevent bleeding episodes and their long-term complications and to arrest bleeding when it occurs.
  • Image not available. Recombinant factor concentrates are usually first-line treatment of hemophilia because they have the lowest risk of infection.
  • Image not available. The goal of therapy for von Willebrand disease is to increase von Willebrand factor and factor VIII levels to prevent bleeding during surgery or arrest bleeding when it occurs.
  • Image not available. Factor VIII concentrates that contain von Willebrand factor are the agents of choice for treatment of type 3 von Willebrand disease and some type 2 von Willebrand disease, and for serious bleeding in type 1 von Willebrand disease.
  • Image not available.Desmopressin acetate is often effective for treatment of type 1 von Willebrand disease. It may also be effective for treatment of some forms of type 2 von Willebrand disease.
  • Image not available. The optimal approach for patients with disseminated intravascular coagulation remains to be determined. The goal of treatment is to diagnose and treat the underlying cause.
  • Image not available. Prophylactic use of phytonadione can effectively prevent vitamin K–dependent bleeding in newborns.

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On completion of the chapter, the reader will be able to:

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  • 1. Describe the regulation of hemostasis and thrombosis.
  • 2. Describe the pathophysiology and genetics of hemophilia and the expected bleeding manifestations based on severity level.
  • 3. List complications of bleeding episodes from hemophilia.
  • 4. Compare the advantages and disadvantages of plasma-derived versus recombinant factor concentrates.
  • 5. Calculate an appropriate factor concentrate dose, given a desired percent correction, for any given factor concentrate product.
  • 6. Formulate treatment options for a patient with hemophilia A who is bleeding and has a high titer inhibitor.
  • 7. Compare and contrast on-demand versus prophylactic administration of factor concentrates.
  • 8. Identify the hematologic disorders for which desmopressin is indicated and the known side effects.
  • 9. Identify strategies to eradicate inhibitors in patients with hemophilia.
  • 10. Determine the appropriate treatment regimen based on a patient's von Willebrand variant.
  • 11. Identify which laboratory tests can aid in the diagnosis of disseminated intravascular coagulation.
  • 12. List etiologies for vitamin K deficiency.
  • 13. Evaluate the various routes of administration for vitamin K.
  • 14. Explain why newborns should receive prophylactic vitamin K.
  • 15. Contrast treatment options for coagulopathies in patients with liver disease.

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A series of complex procoagulant and anticoagulant actions and reactions regulate hemostasis. Maintenance of hemostasis involves the interplay of four major components: (a) the vessel wall, (b) platelets, (c) the coagulation system, and (d) the fibrinolytic system. Coagulation disorders result from an imbalance in the regulation of these components. Bleeding disorders are the result of a decreased number of platelets, decreased function of platelets, coagulation factor deficiency, or enhanced fibrinolytic activity.

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The traditional view of the coagulation cascade consists of intrinsic (propagation), extrinsic, and common pathways where coagulation proteins directed ...

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