Resident Morning Report 12/21 – Hemophilia

Teaching Pearls:

  • Intrinsic Coagulation Pathway
    • XII, XI, IX, VIII
    • Manifested by PTT
  • Extrinsic Coagulation Pathway
    • Factor VII
    • Coumadin decreases production of Factors 2, 7, 9, and 10. Factor VII has the shortest half-life so PT/INR changes are seen first.
    • Manifested by PT/INR
  • Factor VIII Deficiency (Hemophilia A)
    • Genetic
      • X-link recessive trait
        • Present mostly in males at younger age
      • Due to decreased ability to produce factor VIII
      • Elevated PTT due to factor VIII deficiency
      • Treated with prophylactic Factor XIII infusion
      • Clinically presents with hemarthrosis, which can be very severe causing chronic disabling joint disease.
    • Acquired
      • Can be seen in malignancy, post-partum state, or with other autoimmune diseases
      • Can also be idiopathic
      • Elevated PTT due to inhibitor protein against factor VIII
      • Treatment depends on concentration of inhibitor
        • Fast Responder (high inhibitor) – treat with PCC/FFP if actively bleeding, and immunosuppression.
        • Slow Responder (low inhibitor)  – can be treated with factor VIII infusion.
      • Clinical bleeding can manifest in a variety of ways as compared to congenital factor VIII deficiency.
  • Von Willebrand’s Disease (vWF)
    • Most common genetic cause of bleeding.
    • vWF protects factor VIII from being degraded.
      • vWF deficiency can lead to PTT prolongation.
    • vWF binds to a receptor G1b on the platelets to achieve primary hemostasis.
      • vWF disease results in increased bleeding time, which is a result of poor platelet binding and aggregation.

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