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Chapter 54

Chapter 54. Drugs for Hemophilia. Hemophilia. Genetically based bleeding disorder Almost exclusively seen in males 70% of cases result from inheriting defective gene from mother 30% of cases result from spontaneous gene mutation Hemophilia A and hemophilia B

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Chapter 54

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  1. Chapter 54 Drugs for Hemophilia

  2. Hemophilia • Genetically based bleeding disorder • Almost exclusively seen in males • 70% of cases result from inheriting defective gene from mother • 30% of cases result from spontaneous gene mutation • Hemophilia A and hemophilia B • Prognosis is good with good management

  3. Basic Considerations • Pathophysiology • Failure of hemostasis • Normal process • Formation of a platelet plug followed by production of fibrin • Hemophilia • Fibrin production does not proceed normally

  4. Basic Considerations • Inheritance pattern • Recessive gene (both factors VIII and IX) • Carried on the X chromosome • Females can have hemophilia in some cases • Clinical features • Severe hemophilia • Moderate hemophilia • Mild hemophilia

  5. Basic Considerations • Overview of therapy • Pain management • Mild pain: Tylenol • Moderate to severe pain:opioids • Avoid NSAIDs and aspirin • More research needed for COX-2 use • Immunization • Normal immunization schedule • SubQ rather than IM injections • Vaccinated for hepatitis A and B

  6. Fig.54–1. Outline of the coagulation cascade showing clotting factors used to treat hemophilia.

  7. Preparations Used to Treat Hemophilia • Factor VIII concentrates • Production methods and product safety • Plasma-derived factor VIII (low risk of viral contaminants) • Recombinant factor VIII • Adverse effects: allergic reactions • Dosage and administration • On-demand therapy • Prophylactic therapy

  8. Preparations Used to Treat Hemophilia • Factor IX concentrates • Nearly identical pharmacology to that of factor VIII • No risk of HIV/AIDS transmission • Small risk of hepatitis A transmission • On-demand therapy • Prophylactic therapy

  9. Preparations Used to Treat Hemophilia • Desmopressin • Stops or prevents bleeding (mild hemophilia) • Antifibrinolytic agents • Used to prevent recurrent bleeding, less useful to stop ongoing bleeding • Aminocaproic acid (Amicar), tranexamic acid (Cyklokapron)

  10. Managing Patients Who Develop Inhibitors • Inhibitors:antibodies that develop against factor VIII or IX • Drugs for patients with inhibitors • Factor VIII inhibitors • Activated factor VII (factor VIIa) • Anti-inhibitor coagulant complex (AICC) • Porcine factor VIII • Factor IX inhibitors • Limited options • Immune tolerance therapy (ITT) often fails

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