Glanzmann's thrombasthenia is characterized by defective
Glanzmann's thrombasthenia - It is an autosomal recessive disorder due to deficiency/dysfunction of glycoprotein Ilb-IIla (Gp Ilb/IIIa).
Thus there is defective platelet aggregation.
Platelet aggregation is normal to ristocetin(ln constant to Bernard Soulier syndrome) but no aggregation occurs in response to standard agonists (collagen, A DP, thrombin). Other differentiating features include normal platelet count and morphology. BT is prolonged. VWF is normal.
The disease manifests at birth with increased bleeding from umbilical cord stump.
Gp IB/IX
Gp IIB/IIIA
Gp IB/IIIA
Gp IIB/IX