In Glanzman’s thrombasthenia, there is a defect of
**Core Concept**
Glanzmann's thrombasthenia is a rare bleeding disorder characterized by a defect in platelet function, leading to impaired platelet aggregation and clot formation. This condition is caused by a deficiency or dysfunction of specific platelet surface receptors.
**Why the Correct Answer is Right**
The correct answer is related to the glycoprotein IIb/IIIa complex, also known as integrin αIIbβ3. This receptor plays a crucial role in platelet aggregation by binding to fibrinogen, which cross-links platelets and facilitates clot formation. In Glanzmann's thrombasthenia, the deficiency or dysfunction of glycoprotein IIb/IIIa leads to impaired platelet aggregation, resulting in bleeding complications.
**Why Each Wrong Option is Incorrect**
* **Option A:** This option is incorrect because Glanzmann's thrombasthenia is not caused by a defect in von Willebrand factor, which is a different protein involved in platelet adhesion and clot formation.
* **Option B:** This option is incorrect because platelet-derived growth factor (PDGF) is involved in platelet activation and proliferation, but it is not directly related to the pathophysiology of Glanzmann's thrombasthenia.
* **Option C:** This option is incorrect because factor XIII is a transglutaminase that stabilizes blood clots, but it is not directly involved in platelet aggregation or the pathophysiology of Glanzmann's thrombasthenia.
**Clinical Pearl / High-Yield Fact**
Glanzmann's thrombasthenia is an autosomal recessive disorder, meaning that a patient must inherit two defective alleles (one from each parent) to express the condition. This can lead to a range of bleeding complications, including easy bruising, nosebleeds, and prolonged bleeding after surgery or trauma.
**Correct Answer:** C. Glycoprotein IIb/IIIa