Glanzmann's Thrombasthenia -1
Incidence:
GPIIb/IIIa
is missing from platelet membranes
Inherited
defect
Defect:
Rare
Function of GPIIb/IIIa in hemostasis:
Site on which fibrinogen, vWF, fibronectin binds to platelet and allows for aggregation
to occur
Clinical features:
Neonatal
bleeding
Following circumcision
Epistaxis (nose) bleeds
Bleeding from gums