Inherited coagulation disorder –
**Core Concept**
Inherited coagulation disorders refer to a group of bleeding disorders caused by genetic mutations that affect the production or function of clotting factors. These disorders result in impaired blood coagulation, leading to increased bleeding tendency.
**Why the Correct Answer is Right**
Factor VIII deficiency, also known as Hemophilia A, is the most common inherited coagulation disorder. It is caused by a mutation in the F8 gene, which encodes the Factor VIII protein. This protein plays a crucial role in the intrinsic pathway of blood coagulation, and its deficiency leads to impaired clot formation. People with Hemophilia A typically present with spontaneous bleeding into joints and muscles, and they often have a family history of similar bleeding disorders.
**Why Each Wrong Option is Incorrect**
**Option A:** This option is incorrect because Factor V Leiden is an acquired coagulation disorder caused by a mutation in the F5 gene, not an inherited coagulation disorder. It is associated with an increased risk of thrombosis, not bleeding.
**Option B:** This option is incorrect because von Willebrand disease is an inherited bleeding disorder, but it is caused by a deficiency or dysfunction of von Willebrand factor (VWF), not Factor VIII.
**Option C:** This option is incorrect because Protein C deficiency is an inherited coagulation disorder, but it is associated with an increased risk of thrombosis, not bleeding.
**Option D:** This option is incorrect because Antithrombin deficiency is an inherited coagulation disorder, but it is also associated with an increased risk of thrombosis, not bleeding.
**Clinical Pearl / High-Yield Fact**
It's essential to remember that Hemophilia A and Hemophilia B (caused by Factor IX deficiency) are X-linked recessive disorders, meaning they predominantly affect males. Females can be carriers of these disorders, but they are less likely to be affected due to X-chromosome inactivation.
**Correct Answer:** B. von Willebrand disease