Coag 1 (vWF) Flashcards
vWF normally binds to
Factor 8
ADAMTS-13 deficiency
TTP
What test measures the activity of vWF
vWF:Rcof
vWD with all sizes multimers present but decreased
type 1
vWD with no large or medium mulitmers
Type 2a
vWD with no large mulitmers
Type 2b
vWD with almost no mulitmers
Type 3
most common type of vWD
type 1
Type of vWD treated with DDAVP
type 1
Multimers in type 2a vWD
decreased large and medium
Type 1 vWD antigen and cofactor tests
normal Ag (normal quantify) decreased vWF:Rcof (decreased quality)
vWD with increased affinity of HMW multimers to GP1b
type 2b
RIPA in type 2b vWD
increased
Factor 8 is also low in vWD type
type 3
vWD 2M defect
GP1b binding
antigen level and vWF:Rcof in type 2M
normal antigen level
decreased activity
type of vWD where binding to F8 is the problem
type 2N
Labs in vWD type 2N
normal Ag level, normal ristocetin cofactor, normal multimers
defect in platelet type vWD
increased affinity of GP1b for vWF
vWD with abnormal RIPA
2b and platelet type
differentiate between type 2b and platelet type
large multimers present in platelet type
platelet type will aggregate with cryoprecipitate
normal vWF activities by blood group
O 75%
A (100%)
B or AB (120%)
newborn vWF activity
higher than adult
type of vWD to NOT give ddavp to
type 2b
vWD type 2b mutation
gain of function point mutation in GP1b binding domain of vWF
plt type vWD mutation
mutation of plt GP1b protein with enhanced vWF binding
both plt type and 2b clinically cause
clearance ofplts and HMW multimers
type 2M vWD mechanism
prevents binding of vWF tp GP 1b
opp of 2b and plt type
Type 2N vWD mechanism
prevents binding of vWF to F8
vWD that looks like hemophilia
2N and 3
best test to distinguish between 2B and plt-type
in plt-type, plts aggregate with cryoprecipitate
Type Vicenza vWD
increased clearance
still see large multimers on multimer analysis
