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.