Bernard-Soulier Syndrome-2
Disorder it is
confused with:
Composed
of four glycoproteins:
Structure of GPIb/IX:
von
Willebrand disease
GPIba – most frequent mutation
GPIbb
GPIX
GPV – lowest concentration
Treatment:
Platelet
transfusions
Alloantibodies may be formed
Apheresis unit one of choice