Von Willebrand Disease Flashcards
What does a RIPA measure?
The ability of patient VWF to aggregate patient’s own platelets in the presence of ristocetin
Two ways to diagnose Type 1 VWD
Anyone with VWF Ag <30%, regardless of bleeding
VWF Ag 30-50% with bleeding history
What is the defect in Type 1c VWD?
Increased clearance of VWF
Diagnose the VWD: Low VWF Ag, Low VWF:Rco, normal Ag/activity ratio, normal RIPA, normal multimer pattern, normal platelet count
Type 1 VWD
Diagnose the VWD: Decreased VWF Ag, Very low VWF:RCo. Low RIPA, normal platelet count, low of high molecular weight multimers
Type 2A
Diagnose the VWD: Low VWF Ag, very low VWF:RCo, Normal FVIII, normal RIPA, Low platelet count, loss of high molecular weight multimers
Type 2B
Diagnose the VWD: Low VWF Ag, very low VWF:RCo. very low FVIII. Normal RIPA. Normal platelet count. Normal multimers
Type 2N
Diagnose the VWD: Low VWF Ag, very low VWF:RCo, low RIPA, normal platelet count, normal multimer analysis
Type 2M
What is the only type of VWF that desmopressin is useful in treating?
Type 1
What is the difference between plasma-derived and recombinant VWF products?
Plasma derived also contain FVIII (in different ratios)
Side effects to watch out for in desmopressin treatment?
Hyponatremia
Tachyphylaxis
Goal VWF levels in pregnancy for neuraxial anesthesia and delivery
50-150%
What treatment for VWD to avoid in pregnant patients? Why?
DDAVP. Can cause uterine contractions. Also can’t give it to someone getting an epidural because the boluses associated with epidural can precipitate/worsen hyponatremia
Three indications for prophylaxis with VWF products
> 5 bleeding episodes in past 12 months
3 episodes of hemarthrosis at same joint
2 episodes of GI bleed
