Which of the following statements about platelet function defects is true? –
**Core Concept:** Platelet function defects are abnormalities in platelet function that can lead to bleeding diathesis. These defects can be due to deficiencies in platelet production, activation, or aggregation. They are important to recognize as they can cause life-threatening bleeding.
**Why the Correct Answer is Right:** Factor V Leiden is a genetic mutation that results in resistance to the anticoagulant action of activated protein C. This leads to an increased risk of thrombosis, particularly in conditions such as pregnancy, trauma, or surgery. Factor V Leiden is not related to platelet function. Thus, the correct answer is D (Factor V Leiden).
**Why Each Wrong Option is Incorrect:**
A. von Willebrand disease (vWd) is a hereditary disorder affecting platelet function, specifically, the adhesion, secretion, and aggregation of platelets. This option is incorrect because it is a platelet function defect, not related to factor V Leiden.
B. Antiphospholipid syndrome (APS) is an autoimmune disorder where antibodies against phospholipids and/or phospholipid-binding proteins cause arterial and/or venous thrombosis. This option is incorrect because it is related to thrombosis and not platelet function.
C. Hemophilia A is a genetic disorder affecting blood clotting cascade, specifically factor VIII deficiency, causing prolonged bleeding time and increased bleeding tendency. This option is incorrect because it is a coagulation defect, related to clotting factors, not platelet function.
**Clinical Pearl:**
Understanding platelet function defects is essential for recognizing and managing patients with increased bleeding tendencies. In addition to coagulation disorders, platelet function defects should be considered in cases of unexplained bleeding, particularly in settings like pregnancy, trauma, or surgery.
**Correct Answer:** C. Hemophilia A (Factor VIII deficiency)
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A detailed explanation:
**Core Concept:** Hemophilia A is a genetic disorder affecting blood coagulation cascade. It is caused by a deficiency in factor VIII, a protein involved in the intrinsic pathway of blood coagulation. This results in prolonged bleeding time and increased bleeding tendency.
**Why the Correct Answer is Right:** Hemophilia A is a platelet function defect as it affects the coagulation cascade, specifically the intrinsic pathway involving factor VIII. Factor VIII deficiency leads to prolonged bleeding time and increased bleeding tendency.
**Why Other Options are Incorrect:**
A. von Willebrand disease (vWd) is a separate disorder affecting platelet function, specifically von Willebrand factor (VWF) and platelet function. It does not involve the coagulation cascade and is unrelated to factor VIII deficiency.
B. Antiphospholipid syndrome (APS) is an autoimmune disorder causing arterial and venous thrombosis, not platelet function defects. APS is characterized by the production of antiphospholipid antibodies, leading to arterial and venous thrombosis, not factor VIII deficiency or platelet function defects.
C. Hemophilia A (Factor VIII deficiency) is a separate disorder affecting the coagulation cascade, specifically the intrinsic pathway involving factor