<prompt> Which is not true regarding Bernard Soulier syndrome -Ristocetin aggregation is normalAggregation with collagen and ADP is normalLarge plateletsThrombocytopenia  <response>: Bernard Soulier SyndromeAutosomal recessive disorder.Deficiencydysfunction of Gp Ib IX receptorPlatelets cannot adhere to subendothelium because of lack of above receptors for VWF which mediates platelets adhesion.Platelet, aggregation is normal in response to standard agonists collagen, ADP, thrombin but platelets fail to aggregate in response to ristocetin as it acts by a different mechanism.Other laboratory parameters :Thrombocytopenia may be present.Platelets on smear are abnormally large.Bleeding time is abnormally prolonged.VWF factor levels in plasma are normal.Clinical presentation :Recurrent episodes of severe mucosal hemorrhage.Glanzmans thrombastheniaAutosomal recessive disorder.Deficiencydysfunction of Gp IIb IIIa complex.Platelets cannot aggregate because of lack of above receptors for fibrinogen which form bridges between platelets during aggregation.Platelet aggregation is largely abnormal.Platelests fail to aggregate in response to standard agonists collagen, ADP, thrombin as these require fibrinogen binding but aggregate normally to ristocetin as it causes platelet clumping by a different mechanism.Other laboratory parameters :Platelet number is usually normalPlatelet morphology is usually normal..Bleeding time is abnormally prolonged.VWF factor levels in plasma are normal. Clinical presentationRecurrent episodes of severe mucosal hemorrhage. 