Coag 1 (vWF) Flashcards

1
Q

vWF normally binds to

A

Factor 8

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2
Q

ADAMTS-13 deficiency

A

TTP

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3
Q

What test measures the activity of vWF

A

vWF:Rcof

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4
Q

vWD with all sizes multimers present but decreased

A

type 1

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5
Q

vWD with no large or medium mulitmers

A

Type 2a

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6
Q

vWD with no large mulitmers

A

Type 2b

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7
Q

vWD with almost no mulitmers

A

Type 3

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8
Q

most common type of vWD

A

type 1

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9
Q

Type of vWD treated with DDAVP

A

type 1

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10
Q

Multimers in type 2a vWD

A

decreased large and medium

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11
Q

Type 1 vWD antigen and cofactor tests

A
normal Ag (normal quantify)
decreased vWF:Rcof (decreased quality)
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12
Q

vWD with increased affinity of HMW multimers to GP1b

A

type 2b

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13
Q

RIPA in type 2b vWD

A

increased

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14
Q

Factor 8 is also low in vWD type

A

type 3

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15
Q

vWD 2M defect

A

GP1b binding

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16
Q

antigen level and vWF:Rcof in type 2M

A

normal antigen level

decreased activity

17
Q

type of vWD where binding to F8 is the problem

A

type 2N

18
Q

Labs in vWD type 2N

A

normal Ag level, normal ristocetin cofactor, normal multimers

19
Q

defect in platelet type vWD

A

increased affinity of GP1b for vWF

20
Q

vWD with abnormal RIPA

A

2b and platelet type

21
Q

differentiate between type 2b and platelet type

A

large multimers present in platelet type

platelet type will aggregate with cryoprecipitate

22
Q

normal vWF activities by blood group

A

O 75%
A (100%)
B or AB (120%)

23
Q

newborn vWF activity

A

higher than adult

24
Q

type of vWD to NOT give ddavp to

A

type 2b

25
Q

vWD type 2b mutation

A

gain of function point mutation in GP1b binding domain of vWF

26
Q

plt type vWD mutation

A

mutation of plt GP1b protein with enhanced vWF binding

27
Q

both plt type and 2b clinically cause

A

clearance ofplts and HMW multimers

28
Q

type 2M vWD mechanism

A

prevents binding of vWF tp GP 1b

opp of 2b and plt type

29
Q

Type 2N vWD mechanism

A

prevents binding of vWF to F8

30
Q

vWD that looks like hemophilia

A

2N and 3

31
Q

best test to distinguish between 2B and plt-type

A

in plt-type, plts aggregate with cryoprecipitate

32
Q

Type Vicenza vWD

A

increased clearance

still see large multimers on multimer analysis