Coags
Hemophilia A
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Hemophilia A
, Hemophilia, Factor VIII Deficiency
Epidemiology
Most common hereditary
Coagulation Disorder
(1:10,000)
Accounts for 80-90% of Hemophilia cases (remainder are
Hemophilia B
which is a
Factor IX Deficiency
)
Pathophysiology
Sex linked recessive deficiency of factor VIII
Symptoms
Excessive bleeding from slight
Trauma
Dental extraction
Surgery
Visceral bleeding
Hemarthrosis
Contractures
Degenerative
Arthritis
Labs
Partial Thromboplastin Time
(PTT) elevated
ProTime
(INR/PT) normal
Management
Recombinant Factor VIII replacement
Indications
Acute Bleeding
Preoperative prophylaxis
Dosing basic regimen
Typical dosing
Factor VIII 50 units/kg every 8-12 hours OR
Factor VIII (desired concentration - current concentration) * wtKg every 8-12 hours
Factor VIII Infusion
Load: 50 units/kg
Infusion: 4-5 units/hour (directed by Factor VIII levels)
Dosing in major bleeding (directed by involved region and target Factor VIII levels)
Major injury with active bleeding or preoperative and perioperative prophylaxis and management
Target: 100% initially, then 80-100% until wound healed, then 30% of normal Factor VIII Level until
Suture
removal
Recombinant Factor VIII 50 units/kg every 12 hours (adjusted for healing)
Joint bleeding (hemarthrosis)
Target: 80% in acute period, then 40% every other day of normal Factor VIII Level
Recombinant Factor VIII 40 units/kg initially, then 20 units/kg every other day until healed
Gastrointestinal Bleeding
Target: 100% initially, then 50% of normal Factor VIII Level until healed
Recombinant Factor VIII 50 units/kg initially, then 30-40 units/kg daily
Genitourinary bleeding
Target: 100% initially, then 30% of normal Factor VIII Level until healed
Recombinant Factor VIII 50 units/kg initially, then 30-40 units/kg daily
CNS Bleeding
Target: 80-100% initially, then 50-100% of normal Factor VIII Level for 14 days
Recombinant Factor VIII 50 units/kg initially, then 25 units/kg every 12 hours
Oral Bleeding
Target: 100% of normal Factor VIII Level
Recombinant Factor VIII 50 units/kg
Consider topical fibrinolytic agents
Epistaxis
Target: 80-100% initially, then 30% of normal Factor VIII Level until healed
Recombinant Factor VIII 40-50 units/kg then 30-40 units/kg daily
Muscle bleeding
Target: 50% of normal Factor VIII Level
Recombinant Factor VIII 20-40 units/kg daily until healed
Alternative agents when Factor VIII is not available
Cryoprecipitate
10 units
Increases Factor VIII to over 100%
References
Deloughery and Orman in Majoewsky (2013) EM:Rap 13(9): 1-4
DiMichele (1996) Pediatr Clin North Am 43(3): 709-36 [PubMed]
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