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Class notes Patho101
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Von WillebrandDz .
*-vWD is the most
Definition inherited disorder
common
bleeding of
humans
,
adults in the
affecting about 1 %
of
US
.
Bleeding mild
tendency-
·
unnoticed until hemostatic stresses
goes some
revals
·
Autosomal dominant disorder
.
*
Presenting Symptoms spontaneous bleeding memb
. Epistonis
Eg
: --
mucous
from
.
Excessive wounds
bleeding
·
from
Menorrhage
&mot hitogenous
-
· VWD is
clinically
*
Types - Type I
↳ Type
2
Type 3
* VWD associated with
Quantitative defects in VWF.
3 :
Type
-
Type Autosomal dominant dz
·
I : -
mild- moderate .
characterized by dificincy
-
a VWF
>
- 70 % the .
cases
of
- Associated with a
spectrum of mulations-point substitutions
↓
interfere with Maturation of V WF
proteins
Type Autosomal dis.
·
3 :-
>
-
caused
by deletions/frameshift milations
d
little to
resulting in no vWF
synthesis
-
factor
I levels also reduced
sociated
bleeding dis
often
>
-
.
severe
*
Type 2 vWD :
autosomal dominant disorder
.
-
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