15. Treatment of vWD

Treatment of vWD

  • Congenital von Willebrand disease
    • Cryoprecipitate (risk of TTD)
    • Factor replacement
      • Humate P [virally inactivated]
    • DDAVP
  • Acquired von Willebrand disease
    • Treat underlying condition or d/c drugs
    • IV immunoglobulin (IVIG) for lymphoproliferative disease or monoclonal gammopathy (blockade of FcR prevents clearance)
    • Treat with DDAVP to further increase vWF levels
  • Consultation for this patient with laboratory medicine
  • IVIG (1gm/kg) trial
    • 80 gm IV- 5 days before surgery
      • cost $1663
    • Trial of DDAVP given 2 days before surgery
    • DDAVP also given day of surgery

Pre-op Laboratory Studies

vWF AntigenRistocetinBleeding time
2/25/0228%<12%>30 mins
3/22/02 IVIG given
3/25/02138%113%10 mins
DDAVP182%228%6 mins
3/27/02
DDAVP7.5 mins

Outcome
  • Surgery (3/27) went smoothly, with minimal bleeding
  • No further therapy needed; patient d/c'ed to home and no problems on follow-up
  • Approximate cost savings with laboratory consult: $28,000