Coagulation Disorders

von Willebrand Disease

• Decrease in amount/function of von Willebrand protein multimer (vWF)
  • Deficiency of platelet adhesion
• Genetic (Autosomal dominant) or acquired mutation
• factor VIII, factor VIII concentrations may be decreased
  • Increase in PTT - No change in PT
  • von Willebrand multimer also acts as a serum transport protein
• recurrent nosebleeds, easy bruising, petechiae, menorrhagia, gastrointestinal, and gingival bleeding
  • Mucosal bleeding, easy bruising
• ↑ bleeding time, ↑ aPTT (mild), normal platelets
• TX: supportive care, bleeding control, desmopressin (Increase vWF release)

Antithrombin III deficiency

• hypercoagulability caused by deficient antithrombin III
• nephrotic syndrome

Hemophilia

• A and B
  • A - Factor VIII (8) deficiency
  • B - Factor IX (9) deficiency
  • X-linked recessive
    • Males are typically affected
  • Deep tissue bleeding, hemarthroses, muscle bleeds, post-op bleeding
  • ↑ aPTT, normal PT, normal bleeding time
  • TX:
    • A - Mild = Desmopressin, Factor VIII concentrate
    • B - Factor IX concentrate
• C
  • Factor XI (11) deficiency
  • ↑ aPTT (mild)
  • Autosomal recessive (rare)
    • Males and females affected
  • Mild bleeding, after surgery or trauma
  • TX: FFP