Defect of glanzmann’s thrombosthenia is ?
**Core Concept**
Glanzmann's thrombasthenia is a bleeding disorder characterized by the deficiency or dysfunction of platelet glycoproteins IIb/IIIa, leading to impaired platelet aggregation. This defect affects the final common pathway of platelet aggregation, which is mediated by the glycoprotein IIb/IIIa receptor.
**Why the Correct Answer is Right**
The glycoprotein IIb/IIIa receptor plays a crucial role in platelet aggregation by binding fibrinogen and other ligands, facilitating the formation of a platelet plug. In Glanzmann's thrombasthenia, the defective glycoprotein IIb/IIIa receptor fails to bind fibrinogen, resulting in impaired platelet aggregation and bleeding. This defect is due to mutations in the ITGA2B or ITGB3 genes, which encode the alpha IIb and beta 3 subunits of the glycoprotein IIb/IIIa receptor, respectively.
**Why Each Wrong Option is Incorrect**
**Option A:** This option is incorrect because it does not accurately describe the defect in Glanzmann's thrombasthenia. The correct defect is related to the glycoprotein IIb/IIIa receptor, not the von Willebrand factor.
**Option B:** This option is incorrect because it refers to a different bleeding disorder, von Willebrand disease, which is caused by a deficiency or dysfunction of von Willebrand factor.
**Option C:** This option is incorrect because it refers to a different bleeding disorder, platelet dysfunction, which can be caused by various factors, including medications and other conditions.
**Clinical Pearl / High-Yield Fact**
Glanzmann's thrombasthenia is an autosomal recessive disorder, and affected individuals may present with mucocutaneous bleeding, such as nosebleeds and easy bruising. The diagnosis is confirmed by a platelet aggregation study or genetic testing.
**Correct Answer: D. Deficiency or dysfunction of platelet glycoproteins IIb/IIIa**