On completion of the chapter, the reader will be able to:
- 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.
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.
The traditional view of the coagulation cascade consists of intrinsic (propagation), extrinsic, and common pathways where coagulation proteins directed ...