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HEMOPHILIA  A ( factor VIII deficiency)                                 See also  Hemophilia B

A rare hereditary hemorrhagic disorder affecting 1:10,000 MALES

a. Symptom:
It is correlated to the Factor VIII Coagulant Activity Level.

< 1% : bleeding present in first yr of life; has spontaneous hemarthrosis & deep tissue bleeding
1 - 5% : bleeding after trauma in childhood; spontaneous bleeding is rare.
5 - 20% : may present in childhood with hemorrhage after trauma, surgery or dental extraction
20 - 50% : may be undetected until adult life when major trauma or surgery results in severe hemorrhage

b. Management:

  1. Genetic counseling
  2. Avoid aspirin & trauma if possible
  3. Administration of donor screened, heat treated Factor VIII concentrates based on the extent of the disease & bleeding & the location of hemorrhage.
    The calculated Factor VIII concentrate (unit) requirement =
    40 ml/kg x body weight(kg) x 0.25 units/ml (for raising 25% of the Factor VIII level from <1%) as initial bolus; then 1/2 of the initial dose at 6 hrs later, then q12h as long as is needed to control the hemorrhage.
    Each bag of the concentrate has 100 unit.
    For most acute hemathroses of knee, ankle, elbow: 28 36% level
    For serious deep tissue bleeding: raise up to 50% level
    For dental work: up to 50% level + EACA 0.1 g/kg IV bolus, then EACA 0.1 g/kg q6h PO for 10d after the dental work.
    Prior to elective surgery: raise to 5 100% level, then maintain above 50% for the next 10 14d
  4. DDAVP can be used to treat acute traumatic hemorrhage in mild or moderate hemophilics. (Dose IV 0.3 ug/kg in 50 ml salin over 15 30 min)
  5. For some pts with antibody inhibitor against Factor VIII: The amount of Factor VIII concentrate is about 40 unit/kg + an additional 20 units/kg for each Bethesda unit of inhibitor.



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