<prompt> Glanzmanns thrombasthenia is characterized by defectiveGp IBIXGp IIBIIIAGp IBIIIAGp IIBIX  <response>: Glanzmanns thrombasthenia - It is an autosomal recessive disorder due to deficiencydysfunction of glycoprotein Ilb-IIla Gp IlbIIIa.Thus there is defective platelet aggregation.Platelet aggregation is normal to ristocetinln 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. 