TABLE 2

VWD Variants

TestType 1Type 2AType 2BPT-VWDType 2NType 2MType 3
VWF:AgLowLowLowLowLowLowAbsent
VWF:ActLowVWF:Act/VWF:Ag <0.5LowLowLowVWF:Act/VWF:Ag <0.5Absent
FVIIILowNlNlNlLowNlAbsent
RIPANlLowNlNlNlLowAbsent
RIPA-LDAbsentAbsentIncreasedIncreasedAbsentAbsentAbsent
Frequency70%–80%10%–12%3%–5%0%–1%1%–2%1%–2%1%–3%
MultimersNlSmallSmallSmallNlNlAbsent
  • FVIII, factor VIII activity; Nl, normal; PT-VWD, platelet-type pseudo VWD; RIPA, ristocetin-induced platelet aggregation; RIPA-LD, low-dose ristocetin-induced platelet aggregation; VWF:Act, VWF activity; VWF:Ag, VWF antigen. Reprinted with permission from Nichols WL, Hultin MB, James AH, et al. von Willebrand disease (VWD): evidence-based diagnosis and management guidelines, the National Heart, Lung, and Blood Institute (NHLBI) Expert Panel Reports (USA). Haemophilia. 2008;14(2):191.