Glanzmann’s thrombasthenia is characterized by defective
Correct Answer: Gp IIB/IIIA
Description: 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.
Category:
Pathology
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